| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We were then interested in whether the cytoskeletal component , dystrophin related protein ( DRP ; Tinsley , J . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Developmental studies of dystrophin positive fibers in mdx , and DRP localization . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| OBJECTIVES : Genetic depletion of dystrophin related protein ( DRP ) complex causes cardiomyopathy in animals and humans . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin staining was positive but fainter than other DMD muscles . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystroglycan is also expressed in those brain areas where the dystrophin related protein ( utrophin ) is present . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Absence of dystrophin and utrophin in a boy with severe muscular dystrophy . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin , utrophin , and muscular dystrophy . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| For comparison , antibodies against dystrophin and utrophin were also used . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We also localized utrophin , a dystrophin homologue , and laminin , which served as a marker for the basal lamina . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Structure function relationships in dystrophin and utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Low probability of dystrophin and utrophin coiled coil regions forming dimers . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is a dystrophin related cytoskeletal protein expressed in many tissues . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The same repeats are also present in alpha actinin , dystrophin and utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| This protein might correspond to the dystrophin homologue utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We also characterized the expression of utrophin and dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In the adult , it occurs on the crests of the junctional folds , with utrophin , and in the troughs , with dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Comparative analysis of the human dystrophin and utrophin gene structures . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin , like its homologue dystrophin , forms a link between the actin cytoskeleton and the extracellular matrix . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| These hybrid myotubes expressed dystrophin , utrophin and dystrophin associated proteins . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Up regulation of a dystrophin homologue , utrophin , mediates selective DGC retention . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is a paralogue of dystrophin and can functionally replace it in skeletal muscle . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Here we show that mice spermatozoa express only dystrophin Dp 71 and utrophin Up 71 . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Recently , an autosomal homolog of the dystrophin gene ( DMDL ) was identified on chromosome 6q24 . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Chromosome 6 encoded dystrophin related protein ( DRP ) shows significant structural similarities to dystrophin at the carboxyl terminus , though the two proteins are encoded on different chromosomes . ^^^ Both DRP and dystrophin are expressed in muscle and brain and show some similarity in their subcellular localization . ^^^ However , while dystrophin is absent or severely reduced in Duchenne / Becker muscular dystrophy , DRP continues to be expressed . ^^^ Within the brain , dystrophin is enriched at the postsynaptic regions of specific subsets of neurons , while the distribution of DRP is yet to be described . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The distributions of dystrophin , ' dystrophin related protein ' ( DRP ) and beta spectrin were compared with that of acetylcholine receptors ( AChRs ) at rat nerve muscle junctions ( NMJs ) using immunofluorescence techniques . ^^^ In sections , monoclonal antibodies ( MAbs ) to dystrophin and beta spectrin labelled the entire sarcolemma but were concentrated at the NMJs while those to DRP labelled only NMJs . ^^^ In permeabilized muscle fibres , DRP was precisely co localized with the AChRs , whereas the zone of high density labelling of dystrophin and beta spectrin extended 0 . 3 0 . 4 microns beyond the AChRs . ^^^ These data suggest DRP is more closely associated with AChRs than are dystrophin or beta spectrin . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin , the autosomal dystrophin related protein ( DRP ) , is expressed in HeLa cells , smooth muscle like BC3H1 cells from mouse brain , COS monkey kidney cells , the P388D1 monocyte macrophage cell line and untransformed human skin fibroblasts , as well as in rat C 6 glioma and Schwannoma cells . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Here we demonstrate that dystrophin related protein ( DRP , utrophin ) , an autosomal homologue of dystrophin , is associated with an identical or antigenically similar complex of sarcolemmal proteins and that DRP and the dystrophin / DRP associated proteins colocalize to the neuromuscular junction in Duchenne muscular dystrophy and mdx muscle . ^^^ The DRP and dystrophin / DRP associated proteins are found throughout the sarcolemma in small calibre skeletal muscles and cardiac muscle of adult mdx mice . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In the absence of dystrophin in Duchenne muscular dystrophy ( DMD ) patients , DRP is also present in the sarcolemma . ^^^ Dystrophin related protein ( DRP or ' utrophin ' ) is localized in normal adult muscle primarily at the neuromuscular junction . ^^^ DRP is expressed in fetal and regenerating muscle and may play a similar role to dystrophin in early development , although it remains to be determined whether DRP can functionally replace dystrophin in adult tissue . ^^^ To investigate the relationship between DRP and dystrophin in more detail , we have cloned and sequenced the whole DRP cDNA . ^^^ Homology between DRP and dystrophin extends over their entire length , suggesting that they derive from a common ancestral gene . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| To determine whether or not and how dystrophin exists in neuromuscular junctions ( NMJs ) and myotendinous junctions ( MTJs ) , we studied the mid belly and peripheral portions of control and mdx muscles , immunohistochemically and immunoelectrophoretically , using six kinds of polyclonal antibodies , and an antibody against a dystrophin related protein ( DRP ) . ^^^ These data suggest that dystrophin really exists on MTJs , and that dystrophin and DRP exist on NMJs in mouse and human muscles . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin related protein ( DRP ) is an autosomal gene product with high homology to dystrophin . ^^^ We have used highly specific antibodies to the unique C terminal peptide sequences of DRP and dystrophin to examine the subcellular localization and biochemical properties of DRP in adult skeletal muscle . ^^^ DRP is enriched in isolated sarcolemma from control and mdx mouse muscle , but is much less abundant than dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| To examine if this is the translation product of the autosomal transcript with homology to dystrophin mRNA identified by Love et al . ( 1989 ) , we raised an antibody ( PDRP ) against a synthetic peptide corresponding to the putative protein ( DRP ) and examined its expression and cellular localization in human and murine skeletal muscle samples . ^^^ The abundance of DRP in dystrophin deficient muscles might be related to some compensatory mechanisms . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In conclusion , a dystrophin homologue that may be identical to the previously described dystrophin related protein ( DRP ) 1 is expressed in Duchenne muscle with intracellular distribution similar to Xp 21 dystrophin in normal muscle . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We studied the developmental changes of localization of dystrophin and other cytoskeletal proteins , especially actin , spectrin and dystrophin related protein ( DRP ) using immunocytochemistry and quick freezing and deep etching ( QF DE ) method . ^^^ In developmental studies of mouse and human muscle cultures , some myoblasts had positive reactions to spectrin , DRP , and F actin , but not dystrophin . ^^^ In aneurally cultured myotubes , dystrophin , DRP , and spectrin were localized diffusely in the cytoplasm and later in discontinuous patterns on the plasma membrane , when myotubes became mature . ^^^ Spectrin and DRP had more positive reactions in immature myotubes , compared with those of dystrophin . ^^^ In innervated cultured human muscle cells , dystrophin and DRP were localized in neuro muscular junctions , which were co localized with clusters of acetylcholine receptors . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Compared to dystrophin deficient DMD muscle , expression of chromosome 6 encoded dystrophin related protein ( DRP ) was greatly diminished in skeletal muscle of both patients . ^^^ They also suggest a negative correlation between sarcolemmal expression of the dystrophin C terminus and DRP expression at the sarcolemma . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In this recombinant expression system , the dystrophin relatives , human dystrophin related protein ( DRP or utrophin ) and the 87K postsynaptic protein from Torpedo electric organ , also bind to translated beta 1 syntrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| A benign Becker muscular dystrophy ( BMD ) patient with a marked decrease in dystrophin exhibited remarkable expression of dystrophin related protein ( DRP ) on most of the muscle cell membrane . ^^^ Increased DRP expression might compensate for a lack of dystrophin in some BMD patients . . ^^^ A phenotypic Duchenne muscular dystrophy patient with a truncated form of dystrophin exhibited no DRP expression on the muscle cell membrane except for the neuromuscular junction . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| DRP is known to exist in fetal muscles even in mdx mice , an animal model for 10 linked DMD , but not in mature mouse muscles . ^^^ This autosome derived gene product is called dystrophin related protein ( DRP ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| To clarify the localization and characterization of dystrophin and dystrophin related protein ( DRP ) in the brains of normal and mdx mice , we carried out immunostaining and immunoblotting studies using four region specific antidystrophin and anti DRP antibodies . ^^^ Although further studies of brain type dystrophin are necessary , it seems unlikely that DRP participates in any physiological function of the neuronal cells . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunoreactivity of DRP was observed in the vascular walls of both normal and DMD brains , but not in the neuronal cells . ^^^ Compensatory increase of DRP was not noted in DMD brains . ^^^ To clarify the localization and characterization of dystrophin and dystrophin related protein ( DRP ) in the human central nervous system ( CNS ) , we carried out immunoblotting and immunostaining studies using three region specific anti dystrophin and one anti DRP antibodies . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| These results were compared with those from a similar previous study of dystrophin and an autosomal homologue ( utrophin or dystrophin related protein , DRP ) ( Bewick et al . ^^^ These results suggest that at the NMJ , the region of high 43DAG concentration encompasses the area of highest intensity labelling for both DRP and dystrophin . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The respective localizations of dystrophin and dystrophin related protein ( DRP or utrophin ) along the sarcolemmal membrane and at the neuromuscular junctions ( NMJs ) in normal and dystrophin deficient skeletal muscles , were determined using confocal laser microscopy . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Expression of the dystrophin related protein ( DRP or Utrophin ) was examined with Western blot and immunohistochemical methods in diaphragm , limb and also in myoblast transferred muscles of the mdx mouse . ^^^ In limb muscles treated with myoblast transfer , dystrophin positive muscle fibers had no DRP on their surface membrane , although dystrophin negative muscle fibers were DRP positive . ^^^ These findings suggest that excessive expression of DRP is suppressed in the normalized muscle fiber with dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin related protein ( DRP or utrophin ) is an autosomal homologue of dystrophin , a membrane cytoskeletal protein encoded by the Duchenne muscular dystrophy gene . ^^^ In contrast to dystrophin which is predominantly expressed in muscle , DRP is expressed in various tissues . ^^^ In analogy to dystrophin of muscle cells , DRP could be playing an important role in the mechanical stress mechanisms of endothelial cells . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunoblot analysis of dystrophin related protein ( DRP ) . ^^^ Polyclonal antibodies against the carboxy terminal portion of dystrophin related protein ( DRP ) , the putative autosomal gene product which shares sequence homology with dystrophin , show the clear expression of DRP in mouse fetal muscle and in cultured human muscle cells , but not in mature mouse or human muscle . ^^^ DRP has the same molecular mass as 10 linked dystrophin and is recovered from the membrane fraction , but is associated with membranes more loosely than dystrophin . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In Duchenne / Becker dystrophy ( DMD / BMD ) and in polymyositis ( PM ) or dermatomyositis ( DM ) DRP was present throughout the extrajunctional surface membrane of extra and intrafusal muscle fibers , particularly regenerating ones . ^^^ This produced a 15 17 fold increase of DRP over normal in DMD / BMD and 4 10 fold increase over normal in PM and DM on immunoblots . ^^^ The molecular stimulus for the marked upregulation of DRP in DMD / BMD and in the inflammatory myopathies is not known . ^^^ In DMD / BMD the diffuse sarcolemmal DRP may partially compensate for dystrophin deficiency . . ^^^ A dystrophin related protein ( DRP ) encoded by a gene on chromosome 6 was studied in 14 normal and 79 pathological human skeletal muscle biopsies , as well as in cultured myotubes by light microscopic immunocytochemistry and quantitative immunoblots . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Thirty four biopsied muscles of Duchenne , Becker and congenital muscular dystrophy , congenital myotonic dystrophy and amyotrophic lateral sclerosis were examined by an immunocytochemical method with an anti dystrophin related protein ( DRP ) antibody . ^^^ DRP is , therefore , thought to be expressed to compensate for dystrophin deficiency in these muscle fibers . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin related protein ( DRP ) has been reported to exist in the brain , but there are no reports describing its existence in the retina . ^^^ In the present study , DRP localization was examined in rat retinas by immunohistochemistry in comparison to dystrophin localization . ^^^ Dystrophin was observed in the outer plexiform layer , but DRP was recognized in ganglion cell layer and inner nuclear layer , but not in the outer plexiform layer . ^^^ These results suggest that DRP may play a distinct role from that of dystrophin in the rat retina . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Using immunohistochemistry , we find that alpha and beta DG are expressed in the outer plexiform layer of both wild type and mdx retina , where both dystrophin and dystrophin related protein ( DRP ) , but not laminin are present . ^^^ Our results support the hypothesis that alpha and beta DG can interact with dystrophin and DRP in the CNS and perform functions analogous to those of the DGC in muscle . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Characterization of DRP 2 , a novel human dystrophin homologue . ^^^ The currently recognised dystrophin protein family comprises the archetype , dystrophin , its close relative , utrophin or dystrophin related protein ( DRP ) , and a distantly related protein known as the 87K tyrosine kinase substrate . ^^^ We term this class dystrophin related protein 2 ( DRP 2 ) , and suggest that utrophin / DRP be renamed DRP 1 to simplify future nomenclature . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We now present biochemical evidence for an association between utrophin ( dystrophin related protein , DRP ) and a major DAGc component , beta dystroglycan ( 43DAG ) in cultured cell lines which contain little if any dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| To evaluate a potential regulatory role of the nerve , the distribution and expression of dystrophin , of beta dystroglycan ( 43DAG ) and adhalin ( 50DAG ) , two of the dystrophin associated proteins and utrophin ( dystrophin related protein or DRP ) were studied in rat muscles after 2 weeks of denervation . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin , dystrophin related protein ( DRP , utrophin ) and dystroglycan are present not only in muscles but also in the brain . ^^^ Dystrophin is expressed in certain neuronal populations while DRP is associated with perivascular astrocytes . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We demonstrate the use of utrophin / dystrophin related protein ( DRP ) as sensitive control for sample degradation , since it is more labile than dystrophin . ^^^ We suggest that the concomitant or sequential usage of antibodies specific for dystrophin along with utrophin / DRP can help reduce the misdiagnosis of D / BMD . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The correlation between the various proteins and complexes and the dystrophin is known as Dystrophin Associated Glycoproteins ( DAG ) and Dystrophin Related Proteins ( DRP ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| An alternative strategy for DMD therapy , that circumvents many of these problems , has arisen from the demonstration that the DRP utrophin can functionally substitute for the missing dystrophin and its overexpression can rescue dystrophin deficient muscle . ^^^ Dystrophin itself is closely related to three proteins that constitute a family of dystrophin related proteins ( DRPs ) : the chromosome 6 encoded DRP or utrophin , the chromosome 10 encoded , DRP 2 and the chromosome 18 encoded , dystrobrevin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is a chromosome 6 encoded dystrophin related protein ( DRP ) , sharing functional motifs with dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| It has genetic and clinical features in common with humans who carry the gene deletion or mutation of the delta sarcoglycan ( SG ) gene , a component in dystrophin related proteins ( DRP ) . ^^^ CONCLUSIONS : Both the TO 2 hamster and the isoproterenol treated Wistar rat models commonly experience disruption of dystrophin or DRP . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Our results revealed the presence of utrophin ( DRP 1 ) , G utrophin and / or DRP 2 and four dystrophin isoforms ( Dp 427 , Dp 260 , Dp 140 , Dp 71 ) in the normal adult rat retina . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We describe here a comprehensive survey of Drp 2 expression in the mouse by RT PCR , and compare the expression profile of Drp 2 with that of the related genes Dmd , Drp 1 and Dag 1 that encode all the known isoforms of dystrophin , DRP1 / utrophin and a component of the dystrophin associated protein complex , dystroglycan , respectively . ^^^ DRP 2 is predicted to resemble certain short C terminal isoforms of dystrophin and dystrophin related protein 1 ( DRP 1 or utrophin ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| These include a structurally very similar gene in vertebrates encoding utrophin ( DRP 1 ) , which is closely related to dystrophin , and a number of small and simple genes in vertebrates or invertebrates encoding proteins similar to some of the small products of the DMD gene . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The dystrophin related protein , utrophin , is expressed on the sarcolemma of regenerating human skeletal muscle fibres in dystrophies and inflammatory myopathies . ^^^ Utrophin is the 400 kDa protein product of an autosomal homologue ( DMDL ) of the dystrophin gene . ^^^ Utrophin is consistently expressed in all basophilic , regenerating fibres irrespective of the underlying disease or expression of dystrophin . ^^^ These studies do not support the idea that utrophin occupies membrane attachment sites only when dystrophin is absent or reduced , but would be consistent with utrophin expression as part of an activated foetal programme during regeneration . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Muscle biopsy samples from five patients with cytoplasmic body myopathy ( CBM ) were investigated by immunohistochemical ( antibodies to desmin , actin , dystrophin , spectrin , alpha actinin and utrophin ) , immunoelectron microscopic ( antibodies to desmin , actin and dystrophin ) and biochemical ( desmin , dystrophin , actin and utrophin western blots ) methods . ^^^ Using immunofluorescence it was shown that the centers of cytoplasmic bodies ( CB ) were stained by anti actin , anti utrophin and three different anti dystrophin antibodies . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Re evaluation of the distributions of dystrophin and utrophin in sciatic nerve . ^^^ We used specific monoclonal antibodies to fully investigate the presence of utrophin , a dystrophin homologue encoded by a gene located on chromosome 6q24 . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Recent efforts to discover the agrin receptor have led to a surprising conclusion : the only agrin binding component so far detected in muscle cells is dystroglycan , an extracellular protein that is part of the complex of proteins associated with dystrophin , and its homologue , utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is a large cytoskeletal protein which shows high homology to dystrophin . ^^^ In contrast to the sarcolemmal distribution of dystrophin , utrophin accumulates at the postsynaptic membrane of the neuromuscular junction . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin and utrophin : the missing links . ^^^ There is considerable sequence homology between dystrophin and utrophin , both at the protein and DNA level , and consequently it was assumed that their domain structures and functions would be similar . ^^^ We review recent findings and present new hypotheses into the structural and functional properties of the actin binding domain , central coiled coil region and regulatory / membrane protein binding regions of dystrophin and utrophin . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In muscle and nonmuscle tissues , syntrophin is associated with dystrophin , utrophin , and two homologs of the dystrophin carboxy terminal region . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Our data confirm the close evolutionary relationships between the DMD and utrophin loci ; however , the functions for the corresponding proteins probably differ . . ^^^ G utrophin , the autosomal homologue of dystrophin Dp 116 , is expressed in sensory ganglia and brain . ^^^ The utrophin gene is closely related to the dystrophin gene in both sequence and genomic structure . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin , or dystrophin related protein , is an autosomal homologue of dystrophin . ^^^ Since utrophin has a similar domain structure to dystrophin it has been suggested that it could substitute for dystrophin in dystrophic muscle . ^^^ Like dystrophin , utrophin has been shown to be associated with a membrane bound glycoprotein complex . ^^^ The expressed NH 2 terminal 261 amino acid domain of utrophin has an affinity for skeletal F action ( Kd 19 + / 2 . 8 microM ) , midway between that of the corresponding domains of alpha actinin ( Kd 4 microM ) and dystrophin ( Kd 44 microM ) . ^^^ These data ( together with those of Matsumura et al . ( 1992 ) Nature , 360 , 588 591 ) demonstrate for the first time that utrophin is capable of performing a functionally equivalent role to that of dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In contrast to normal cultures , the utrophin content of long term dexamethasone treated DMD myotube cultures was significantly greater than that of the corresponding untreated cultures . ^^^ Utrophin mRNA transcript levels normalized to total poly ( A ) were unaffected by dexamethasone treatment of either normal or DMD myotube cultures , suggesting the effect of dexamethasone on utrophin accumulation by DMD cultures is mediated post transcriptionally . ^^^ A combination of an increase in myotube numbers and lack of competition with dystrophin for membrane binding sites in DMD myotubes may explain the distinct effects of dexamethasone on utrophin levels in normal and DMD cultures . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The cytoskeletal proteins utrophin , dystrophin and alpha actinin are predicted to form antiparallel dimers thus potentially bringing their NH 2 terminal F actin binding domains in close proximity to their EF hand containing COOH terminal domains . ^^^ These findings have implications for the structural organisation of utrophin dimers and also for the replacement of dystrophin by over expression of utrophin in dystrophic muscle . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Expression of utrophin ( dystrophin related protein ) during regeneration and maturation of skeletal muscle in canine 10 linked muscular dystrophy . ^^^ The regulation of utrophin , the autosomal homologue of dystrophin , has been studied in the canine 10 linked model of Duchenne muscular dystrophy . ^^^ To establish whether this abnormal presence of utrophin in dystrophic muscle is a consequence of continued expression following regeneration , or is attributable to a disease related up regulation , the expression of utrophin was compared immunocytochemically with that of dystrophin , beta spectrin and neonatal myosin in regenerating normal and dystrophic canine muscle , following necrosis induced by the injection of venom from the snake Notechis iscutatis . ^^^ In normal regenerating muscle , sarcolemmal utrophin and dystrophin were detected concomitantly from 2 3 d post injection , prior to the expression of beta spectrin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The syntrophins are a multigene family of proteins which bind C terminal domains of dystrophin , utrophin and homologs thereof . ^^^ Anti alpha 1 syntrophin antibodies gave strong labeling of the sarcolemma and NMJ in normal rat and mouse muscle , and similar but much weaker labeling in dystrophin minus mdx muscle . beta 2 Syntrophin therefore appears to be specific to the NMJ in normal muscle , as is utrophin , and may be involved in acetylcholine receptor clustering . alpha 1 Syntrophin appears to be associated mainly with dystrophin , as expected , but a small portion must be associated with another protein , possibly homologs of the electric tissue 87K protein . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Autosomal recessive distal muscular dystrophy : normal expression of dystrophin , utrophin and dystrophin associated proteins in muscle fibers . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Full length and short forms of utrophin , the dystrophin related protein . ^^^ All previous studies of the localization of utrophin ( the dystrophin related protein ) in muscle and other tissues have been performed only with antibodies against the C terminal region of the protein . ^^^ Since several short forms of dystrophin , the apo dystrophins , are produced from the 3 ' end of the dystrophin gene , there is a possibility that similar short forms of utrophin exist and that these could be responsible for some of the many different localizations of ' utrophin ' in muscle . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Expression of dystrophin associated glycoproteins and utrophin in carriers of Duchenne muscular dystrophy . ^^^ The expression of dystrophin , the dystrophin associated proteins and utrophin has been studied immunocytochemically in three young , manifesting carriers of Duchenne muscular dystrophy , aged 3 , 5 and 12 yrs , one adult manifesting carrier , aged 60 yrs , and one presumptive carrier with a raised serum creatine kinase , aged 24 yrs , the mother of the 5 yr old manifesting carrier . ^^^ Utrophin was detected on the sarcolemma of fibres both with and without dystrophin and the dystrophin associated proteins . ^^^ Thus , dystrophin and utrophin are co expressed in several fibres in carriers . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Morphological , immunobiochemical , immunocytochemical analyses and contraction studies of these cells demonstrated that ( 1 ) the cell cytoskeleton in mdx mice is not affected by the absence of dystrophin since proteins such as caldesmon , a actin , and vinculin are expressed similarly in normal mice , ( 2 ) utrophin ( or dystrophin related protein ) overexpression does not compensate for the physiological and functional role of the lacking dystrophin . ^^^ These data suggested that dystrophin and utrophin can not substitute one another and may play different or complementary roles within smooth muscle cells . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Identification of alpha syntrophin binding to syntrophin triplet , dystrophin , and utrophin . ^^^ However , similar to utrophin , alpha syntrophin is only present at the neuromuscular junction in mdx mouse muscle in which dystrophin is absent . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Association of aciculin with dystrophin and utrophin . ^^^ In contrast , utrophin , an autosomal homologue of dystrophin , was not codistributed with aciculin in muscle cell cultures and in skeletal muscle tissues . ^^^ Whereas dystrophin was shown to be a major aciculin associated protein in skeletal muscle , immunoblotting of anti aciculin immunoprecipitates with antibodies against utrophin showed that aciculin is associated with utrophin in cultured A7r5 smooth muscle cells and REF 52 fibroblasts . ^^^ Taken together , our data show that aciculin is a novel dystrophin and utrophin binding protein . ^^^ Association of aciculin with dystrophin ( utrophin ) in various cell types might provide an additional cytoskeletal matrix transmembrane link at sites where actin filaments terminate at the plasma membrane . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunostaining of biopsied skeletal muscle of 4 Duchenne ( DMD ) , 12 Becker muscular dystrophy ( BMD ) and 3 DMD carriers ' was performed using monoclonal antibodies against dystrophin and utrophin . ^^^ In DMD , the utrophin positive fibers corresponded to dystrophin negative fibers . ^^^ In DMD carriers , a cluster of dystrophin negative fibers which was positive for utrophin were prominent . ^^^ Immunostaining of dystrophin and utrophin in skeletal muscle of dystrophinopathies . ^^^ In dystrophinopathy , the immunostaining of dystrophin and utrophin is useful , in combination with dystrophin gene analysis to make a definite diagnosis . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Blots also showed an increase ( 143 % ) in the dystrophin related protein called utrophin , another myotendinous junction constituent , which may be able to substitute for dystrophin directly . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunohistochemistry revealed labelling of the inclusions for desmin , dystrophin and vimentin , but not for alpha actinin , spectrin , utrophin or myosin heavy chains . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The expression of the 43 kDa dystrophin associated glycoprotein ( 43DAG ) has been studied using immunohistochemical labelling with a monoclonal antibody , MANDAG 1 , and compared with immunolabelling for dystrophin and the dystrophin related protein , utrophin , in normal muscle and in muscle from 50 patients with neuromuscular disease . 43DAG and dystrophin were expressed in vascular smooth muscle and at the sarcolemma of normal muscle fibres , with increased labelling at neuromuscular and myotendinous junctions . 43DAG expression was reduced in Duchenne and Becker dystrophies with patchy labelling , more intense around presumptive satellite cells . ^^^ In Becker dystrophy , 43DAG expression was more extensive around individual fibres , showed more interfibre variation and was more closely related to the intensity of immunolabelling for both dystrophin and utrophin than in Duchenne dystrophy . ^^^ Utrophin may confer some additional stability to the membrane integration of 43DAG but this is incomplete where dystrophin is absent or abnormal . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Deficiency of the 50 kDa dystrophin associated glycoprotein and abnormal expression of utrophin in two south Asian cousins with variable expression of severe childhood autosomal recessive muscular dystrophy . ^^^ Abnormal expression of utrophin , the dystrophin related protein , was observed on the surface of several non regenerating muscle fibres , with less intense immunolabelling in the clinically more affected child . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| These results in mdx mice along with observed utrophin expression in severely affected DMD patients suggest that overexpression of utrophin is not enough to explain the stability of regenerated fibers in mdx mice . . ^^^ Does utrophin expression in muscles of mdx mice during postnatal development functionally compensate for dystrophin deficiency . ^^^ Utrophin expression is detected by dystrophin / utrophin cross reacting antibodies and can only be evaluated in mdx mouse muscles ( in absence of dystrophin ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin localization in normal and dystrophin deficient heart . ^^^ Utrophin ( or dystrophin related protein ) , a dystrophin homologous protein , was found to be expressed in fetal muscles and reexpressed in dystrophin deficient skeletal muscle fibers . ^^^ We therefore examined utrophin expression in normal and in dystrophin deficient hearts . ^^^ METHODS AND RESULTS : The expression and subcellular distribution of utrophin was examined in cardiac muscle by immunoblot and immunofluorescence analysis in normal bovine heart compared with dystrophin . ^^^ Utrophin expression was also examined in normal and dystrophin deficient hearts of MDX mice . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We found that , although the amount of GPC was reduced in DMD muscles where utrophin but not dystrophin was distinctly present , 43DAG ( A3a ) was fairly heavily and 50DAG ( A 2 ) was lightly but distinctly stained on the cell surfaces . ^^^ Therefore , it is likely that 43DAG ( A3a ) is essential for the fixation of utrophin to cell membranes , as in the case of dystrophin . 50DAG ( A 2 ) may play other important roles in the pathogenesis of DMD . . ^^^ Expression of utrophin ( dystrophin related protein ) and dystrophin associated glycoproteins in muscles from patients with Duchenne muscular dystrophy . ^^^ It is likely that the capability of utrophin to preserve 50DAG ( A 2 ) is less than that of dystrophin , although utrophin has been reported to bind to GPC . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The tissue specific expression of 59 1 DAP mRNA , which is most prominent in skeletal and cardiac muscle and is also detected in brain , parallels that of dystrophin but not of utrophin . ^^^ However , in mdx mouse cardiac muscle , the up regulation of utrophin preserves all dystrophin associated proteins except 59 DAP . ^^^ Our results suggest that the 59 DAP triplet may contain different protein species and that the 59 1 DAP may associate more specifically with dystrophin than with utrophin . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The utrophin ( UTRN ) locus is the autosomal homologue of the DMD ( Duchenne muscular dystrophy ) gene and encodes a protein , utrophin which is thought to be upregulated in the absence of dystrophin . ^^^ Expression of the dystrophin related protein ( utrophin ) gene during mouse embryogenesis . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| With the antibody against the C terminal domain of dystrophin , we found three bands other than the 400 kDa band corresponding to dystrophin or utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Antibodies against dystrophin , utrophin and DAGs including 50DAG ( A 2 ) , 43DAG ( A3a ) and 35DAG ( A 4 ) were employed for the examination . ^^^ Dystrophin was stained strongly and utrophin stained very faintly along the sarcolemma of the dystrophic hamster , similar to the control . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| If this is the case , it may be possible to modify the regulation of utrophin expression as an alternative route to dystrophin gene therapy for sufferers of DMD and / or BMD . . ^^^ Utrophin : a potential replacement for dystrophin . ^^^ This paper reviews the evidence that utrophin , the autosomally encoded protein related to dystrophin , may be capable of performing the same cellular functions as dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Here we demonstrate the co localization of Dp 116 ( a 116 kDa protein product of the DMD gene ) , full size utrophin , alpha and beta dystroglycan , 59DAP and 35DAG in a thin rim surrounding the outermost layer of myelin sheath of peripheral nerve fibers . ^^^ Differential expression of dystrophin , utrophin and dystrophin associated proteins in peripheral nerve . ^^^ In neuromuscular junctions , utrophin , an autosomal homologue of dystrophin , is associated with sarcolemmal proteins identical or immunologically homologous to the dystrophin associated proteins . ^^^ Our results demonstrate the varied expression of the components of the dystrophin / utrophin glycoprotein complex between skeletal muscle and peripheral nerve suggesting the complex may exist in varied compositions and have varied functions in these two tissues . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We examined muscle biopsies from patients with Duchenne muscular dystrophy ( DMD : 39 patients ) and Becker muscular dystrophy ( BMD : 11 patients ) , female DMD carriers ( 4 patients ) , and control subjects ( 26 persons ) for the expression of dystrophin and utrophin . ^^^ Reciprocal expression of dystrophin and utrophin in muscles of Duchenne muscular dystrophy patients , female DMD carriers and control subjects . ^^^ On the other hand , muscles from DMD patients showed the inverse staining patterns : dystrophin was negative and utrophin staining strong . ^^^ We consider that utrophin may have a function similar to that of dystrophin , and compensate to some extent for dystrophin deficiency in DMD . . ^^^ Control subjects showed all fibers to be dystrophin positive , while utrophin staining was negative or weak . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin and dystrophin are highly homologous proteins which are reciprocally expressed in DMD ( Duchenne muscular dystrophy ) muscle . ^^^ The remarkable similarity of these proteins suggests that they may play a similar cellular role in some circumstances ; if this were the case then utrophin may be capable of replacing dystrophin in DMD patients . ^^^ The utrophin and dystrophin genes share similarities in genomic structure . ^^^ In this paper we show that the genomic structure of the utrophin gene is similar to the dystrophin gene , further exemplifying the relatedness of the two genes and their gene products . ^^^ In contrast to dystrophin , the utrophin gene has a long 5 ' untranslated region composed of two exons and a cluster of unmethylated , rare cutting restriction enzyme sites at the 5 ' end of the gene . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin ( a dystrophin related protein ) is an ubiquitous protein whose role is still unclear . ^^^ We investigated three properties of cultured rat aortic smooth muscle cells : morphology , contractile ability , and expression of dystrophin , utrophin , h CaD , and 1 CaD . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Association of utrophin and multiple dystrophin short forms with the mammalian M ( r ) 58 , 000 dystrophin associated protein ( syntrophin ) . ^^^ Using immunoaffinity purifications from various rat tissues and immunoblotting , we find that syntrophin associates with dystrophin , utrophin ( the chromosome 6 encoded dystrophin homolog formerly known as dystrophin related protein ) , multiple proteins which are cross reactive with 87K , and two subfamilies of 71K like proteins ( CRCT containing proteins encoded by the dystrophin gene under the control of an alternative promoter in intron 62 ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| This protein was then used to prepare two monoclonal antibodies ( mAbs ) which react with native dystrophin on frozen muscle sections and with denatured dystrophin on western blots but which do not cross react with the dystrophin related protein , utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The importance of using protein specific antibodies for dystrophin analysis has been underscored by the identification of a protein , designated utrophin , which exhibits significant sequence homology with dystrophin . ^^^ This review addresses the recent studies of dystrophin and utrophin expression in an attempt to illustrate the transcriptional diversity of these large genes and the localization of their protein products within various tissues . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Sequence analysis of the entire cDNA for the autosomal dystrophin related protein utrophin has shown that dystrophin and utrophin are closely related . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin related protein ( utrophin ) , an autosomal homologue of dystrophin , was studied in skeletal muscle from normal fetuses aged 9 26 weeks and one stillbirth of 41 weeks ' gestation , and compared with low and high risk DMD fetuses aged 9 20 weeks . ^^^ Samples were not available to determine whether or when , utrophin levels decline in DMD fetal muscle . ^^^ Dystrophin related protein , utrophin , in normal and dystrophic human fetal skeletal muscle . ^^^ Dystrophin related protein ( utrophin ) , an autosomal homologue of dystrophin , was studied in skeletal muscle from normal fetuses aged 9 26 weeks and one stillbirth of 41 weeks ' gestation , and compared with low and high risk DMD fetuses aged 9 20 weeks . ^^^ Utrophin is therefore expressed in the presence and absence of dystrophin and down regulated before birth in normal fetal muscle fibres . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Monoclonal antibodies targeted against the C terminal domain of dystrophin or utrophin . ^^^ The structure function relationships of dystrophin , a protein which is absent or defective in patients with Duchenne or Becker muscular dystrophies , and utrophin can only be compared if specific antibodies are produced . ^^^ We expressed C terminal parts of dystrophin and utrophin in expression vectors . ^^^ We observed antibody specificity towards 400 kDa dystrophin and / or utrophin protein bands , either by Western blot analysis or immunodetection in human skeletal ( quadriceps ) and smooth ( uterus ) muscles . ^^^ These antibodies have been used to compare the relative abundance of both dystrophin and utrophin relative to the structures analyzed . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In order to detect an inverse correlation of utrophin presence and clinical severity , we have assessed utrophin distribution and quantity in DMD and Becker ( BMD ) patients of different ages and stages of clinical severity . ^^^ On Western blot , utrophin bands with concentrations 2 to 10 fold greater than in normal controls were detected in all DMD / BMD patients . ^^^ In a DMD patient with growth hormone ( GH ) deficiency and a BMD like clinical course , utrophin levels were comparable to the other typical DMD cases , which reinforces the hypothesis that the observed increase in utrophin is apparently not responsible for a milder clinical course in some patients with Xp 21 muscular dystrophies . . ^^^ However , the observation that utrophin is maintained in the extrajunctional plasma membrane in Duchenne ( DMD ) and in mdx muscle fibers has led to the suggestion that excess utrophin might compensate for dystrophin deficiency in the Xp 21 muscular dystrophies . ^^^ However , no negative correlation was found between the amount of utrophin and the severity of clinical course , implying that the detectable utrophin levels in these patients did not compensate for dystrophin deficiency . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin , a protein encoded by chromosome 6 is highly homologous to the cysteine rich domain and most of the C terminal domain of dystrophin . ^^^ There was no correlation between utrophin expression and progressive dystrophin membrane localization . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Presence of long and short dystrophin and / or utrophin products in Torpedo marmorata peripheral nerves . ^^^ Western blot analyses of Torpedo marmorata peripheral nerve extracts revealed the existence of three proteins belonging to the dystrophin family : a M ( r ) 400 kDa protein band detected with dystrophin / utrophin , dystrophin specific and Torpedo utrophin specific antibodies , a molecule identified as Dp 116 and , for the first time at the protein level , a new protein probably corresponding to Up 116 . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In mdx embryos the pattern of distribution of dystroglycan mRNA remains unchanged , as do those of utrophin and apo dystrophin mRNAs . ^^^ This observation implies that the observed changes in the relative abundance of DAGs and utrophin in dystrophin deficient muscle occur post transcriptionally . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We demonstrate that ( 1 ) alpha dystroglycan is an extracellular peripheral membrane glycoprotein that links beta dystroglycan in the Schwann cell outer membrane with laminin 2 in the endoneurial basal lamina , and ( 2 ) dystrophin homologues Dp 116 and utrophin are cytoskeletal proteins of the Schwann cell cytoplasm . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| A novel dystrophin / utrophin associated protein is an enzymatically inactive member of the phosphoglucomutase superfamily . ^^^ A 60 kDa protein localised in adherens type cellular junctions , and previously called aciculin , has been found to interact with the cytoskeletal proteins dystrophin and utrophin [ Belkin , A . ^^^ The significance of the interaction between dystrophin / utrophin and an increasing number of cytoplasmic proteins including PGM RP remains to be explored . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The dystrophin homolog utrophin was detectable only in the sarcolemmal membrane and was absent from the myofibrils as were other sarcolemmal glycoproteins such as adhalin and the sodium calcium exchanger . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The frequency of patients with 50 kd dystrophin associated glycoprotein ( 50DAG or adhalin ) deficiency in a muscular dystrophy patient population in Japan : immunocytochemical analysis of 50DAG , 43DAG , dystrophin , and utrophin . ^^^ To elucidate the frequency of patients having the 50DAG deficiency in a muscular dystrophy population in Japan , we immunocytochemically examined 50DAG , 43DAG , dystrophin , and utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| It shows extensive sequence similarity to dystrophin leading to postulation that utrophin may be able to compensate for the absence of dystrophin in Duchenne muscular dystrophy ( DMD ) patients . ^^^ In order to study the transcriptional control of utrophin expression including its regulation at the neuromuscular junction , and as a first step in the development of a potential DMD therapy , we have cloned the utrophin promoter region from human and mouse . ^^^ This study provides the basis for the understanding of the regulatory mechanism that controls utrophin expression and provides the data needed to develop methods for the upregulation of utrophin in DMD patients . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Computer assisted analysis at the protein level revealed a 56 amino acid domain with homologies of approximately 40 % with a sequence bordering the actin binding domains of dystrophin , utrophin , beta spectrin and alpha actinin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We purified the nicotinic acetylcholine receptor from digitonin solubilized rabbit skeletal muscle by affinity chromatography and detected many proteins linked to AChR , including dystrophin , adhalin , beta dystroglycan , utrophin , rapsyn , and actin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The precise localization and semiquantitative correlation of dystrophin , utrophin and beta dystroglycan expression on the sarcolemma of skeletal muscle cells obtained from patients with Becker muscular dystrophy ( BMD ) was studied using three types of double immunofluorescence . ^^^ These results suggest that utrophin may compensate for dystrophin deficiency found in BMD by binding to beta dystroglycan . . ^^^ Dystrophin , utrophin and beta dystroglycan expression in skeletal muscle from patients with Becker muscular dystrophy . ^^^ The staining intensities of dystrophin and utrophin were approximately reciprocal at sarcolemmal sites where dystrophin expression was obviously observed . ^^^ The staining intensity of beta dystroglycan was strong in areas where dystrophin staining was also strong and utrophin expression was weak . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Characterisation of the dystrophin related protein utrophin in highly purified skeletal muscle sarcolemma vesicles . ^^^ Due to its restricted localisation to the neuromuscular junction and based on sequence homology to cytoskeletal proteins , the dystrophin related protein utrophin is thought to be an important constituent of the membrane cytoskeleton of the postsynaptic muscle membrane and may be involved in the clustering of acetylcholine receptors at the neuromuscular junction . ^^^ Therefore , utrophin appears to be a specialised isoform which performs the membrane cytoskeletal function ( s ) of dystrophin at the postsynaptic membrane of the neuromuscular junction . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunohistochemical study of utrophin and dystrophin at the motor end plate in myasthenia gravis . ^^^ We studied the densities of utrophin and dystrophin at the motor end plates of patients with myasthenia gravis ( MG ) using immunohistochemical analysis . ^^^ We conclude that , at the motor end plate , utrophin may be more closely associated than dystrophin with the acetylcholine receptor , and that it plays a different role . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin , utrophin and the dystrophin associated glycoproteins , beta dystroglycan and adhalin , were analyzed , together with the membrane cytoskeletal proteins beta spectrin , vinculin and talin , and adult and fetal myosin heavy chains , in 25 normal human fetuses from 8 to 24 weeks of gestation . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Binding of agrin to its receptor , alpha dystroglycan , is followed by rearrangements of the muscle membrane cytoskeleton with localized replacement of dystrophin by utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Spatial relationships of utrophin , dystrophin , beta dystroglycan and beta spectrin to acetylcholine receptor clusters during postnatal maturation of the rat neuromuscular junction . ^^^ We have followed the changes during this period in the distribution of four proteins associated with the postsynaptic cytoskeleton at mature neuromuscular junctions ( utrophin , dystrophin , beta dystroglycan and beta spectrin ) to see if any of them co localizes with acetylcholine receptors during the remodelling process , as would be required if it serves to link acetylcholine receptors to the cytoskeleton . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is an autosomally encoded homologue of dystrophin , the protein product of the Duchenne muscular dystrophy ( DMD ) gene . ^^^ However , during development , and in some myopathies including DMD , utrophin is also found at the sarcolemma . ^^^ Utrophin : a structural and functional comparison to dystrophin . ^^^ Although , utrophin is very similar in sequence to dystrophin and possesses many of the protein binding properties ascribed to dystrophin , both proteins are expressed in an apparently reciprocal manner and may be coordinately regulated . ^^^ In normal skeletal muscle , utrophin is found at the neuromuscular junction ( NMJ ) whereas dystrophin predominates at the sarcolemma . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We investigated the expression of the DAPs beta dystroglycan , alpha sarcoglycan , gamma sarcoglycan and syntrophin as well as utrophin in the muscles of 13 Duchenne muscular dystrophy ( DMD ) carriers ( with variable percentages of dystrophin deficient fibers and with a range of clinical symptoms ) , 2 Becker muscular dystrophy ( BMD ) carriers ( expressing a highly truncated protein in some fibers ) , 2 girls with a DMD like phenotype , and 11 BMD carriers with almost normal dystrophin expression ( reduced or patchy distribution in a few fibers only and rare dystrophin deficient fibers ) . ^^^ Utrophin expression was slightly increased in a proportion of fibers in the DMD and BMD carriers with dystrophin mosaicism . ^^^ We conclude that absence or reduction of dystrophin in muscle fibers of DMD and BMD carriers causes a reduction of DAPs in the same fibers , as observed in DMD and BMD patients , while utrophin does not seem to play a role in DAP expression in adult muscle . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| These data suggest that systemic upregulation of utrophin in DMD patients may lead to the development of an effective treatment for this devastating disorder . . ^^^ One alternative approach to treatment would be to upregulate the closely related protein , utrophin , which might be able to compensate for the dystrophin deficiency in all relevant muscles . ^^^ As a first step to this approach , we have expressed a utrophin transgene at high levels in the dystrophin deficient mdx mouse . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Cloning and expression of full length mouse utrophin : the differential association of utrophin and dystrophin with AChR clusters . ^^^ The expression of recombinant utrophin is compared with that of its homologue , dystrophin ( 427 kDa ) . ^^^ We demonstrate that recombinant utrophin is targeted into agrin induced acetylcholine receptor ( AChR ) clusters , while recombinant dystrophin is evenly distributed along cell membranes in cultured Sol 8 muscle cells . ^^^ This observation suggests that utrophin and dystrophin may interact with different cytoskeletal proteins . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In BMD patients , utrophin was faintly expressed in the arrector pili muscles in 2 cases , and negative in the other 5 patients . ^^^ Immunostaining of utrophin was positive with variable intensity in the arrector pili muscles in all DMD patients . ^^^ On the contrary , in the arrector pili smooth muscle utrophin is not expressed in normal controls but it is in dystrophinopathies , paralleling the findings in striated muscle , which expresses utrophin in a reciprocal manner with respect to dystrophin . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is a large cytoskeletal protein that is homologous to dystrophin , the protein mutated in Duchenne and Becker muscular dystrophy . ^^^ In skeletal muscle , dystrophin is broadly distributed along the sarcolemma whereas utrophin is concentrated at the neuromuscular junction . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Laminin induced clusters also contained dystrophin , but unlike agrin induced clusters , they did not contain acetylcholine receptors , utrophin , or phosphotyrosine , and their formation was not inhibited by a tyrosine kinase inhibitor . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Myotubes expressing the utrophin and dystrophin DGC binding domain formed significantly fewer acetylcholine receptor clusters in response to agrin than myotubes expressing other proteins . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Recently , the use of a transgenic mouse model system for Duchenne muscular dystrophy has demonstrated the ability of utrophin to functionally replace dystrophin and alleviate the muscle pathology ( see Tinsley , J . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In contrast when using the dystrophin / utrophin monoclonal H ' 3E7 antibody , we found a clear difference between rabbit and T . marmorata peripheral nerves according to fluorescent labeling detected within Torpedo nerve axons . ^^^ Further differences were noted following western blot analyses of T . marmorata peripheral nerve extracts , highlighting the presence of a new and specific M ( r ) 70 kDa protein band belonging to the dystrophin family , which is localized within axons in addition to : ( 1 ) an M ( r ) 400 kDa protein band detected with dystrophin / utrophin antibodies ; and ( 2 ) an M ( r ) 116 kDa doublet protein band corresponding to Dp 116 and Up 116 isoforms . ^^^ All of these products , detected according to the specificities of the monoclonal antibodies used , are discussed in terms of their potential identities as short and long dystrophin or utrophin mammalian products . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Different utrophin and dystrophin properties related to their vascular smooth muscle distributions . ^^^ Monoclonal antibodies used to distinguish between dystrophin and utrophin were systematically applied to skeletal muscles containing arteries and veins . ^^^ Small arteries were found to contain long forms of both utrophin and dystrophin , while small veins contained only long forms of utrophin . ^^^ Regardless of their tissue distributions , we assumed that each of these molecules had distinct properties , i . e . dystrophin with a mechanical function and utrophin with an architectural function . ^^^ This difference in the roles of dystrophin and utrophin could reduce the efficiency of protection against muscle membrane degeneration when utrophin overexpression is programmed . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The sarcolemmal distribution of alpha 1 and beta 1 syntrophins suggests association with dystrophin and dystrobrevin , whereas all three syntrophins could potentially associate with utrophin at the neuromuscular junction . ^^^ Utrophin complexes immunoisolated from skeletal muscle are highly enriched in beta 1 and beta 2 syntrophins , while dystrophin complexes contain mostly alpha 1 and beta 1 syntrophins . ^^^ Since individual syntrophins do not have intrinsic binding specificity for dystrophin , dystrobrevin , or utrophin , the observed preferential pairing of syntrophins must depend on extrinsic regulatory mechanisms . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin dystrophin deficient mice as a model for Duchenne muscular dystrophy . ^^^ We describe mice deficient for both dystrophin and the dystrophin related protein utrophin . ^^^ The data suggest that utrophin and dystrophin have complementing roles in normal functional or developmental pathways in muscle . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin : a model for Duchenne muscular dystrophy . ^^^ Utrophin , a homolog of dystrophin , is confined to the postsynaptic membrane at skeletal neuromuscular junctions and has been implicated in synaptic development . ^^^ Thus , utrophin attenuates the effects of dystrophin deficiency , and the double mutant may provide a useful model for studies of pathogenesis and therapy . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Although the precise function of utrophin at the postsynaptic membrane of the neuromuscular junction still remains unclear , despite recent genetic ' knockout ' experiments , a separate study in a transgenic mouse model system for Duchenne muscular dystrophy ( DMD ) has nonetheless shown that overexpression of utrophin into extrasynaptic regions of muscle fibers can functionally compensate for the lack of dystrophin and alleviate the muscle pathology . ^^^ In this context , the next step is to identify the mechanisms presiding over expression of utrophin at the neuromuscular synapse in attempts to induce its expression throughout DMD muscle fibers . ^^^ Identification of the events culminating in the transactivation of the utrophin gene within synaptic myonuclei should provide important cues for the development of an effective therapeutic strategy for DMD . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In each patient , immunolabelling of the neuromuscular junction for rapsyn , dystrophin , beta dystroglycan and a form of beta spectrin was strong but that for utrophin was markedly reduced or absent . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The thrust of research during the past 18 months has focused on two approaches : adenovirus mediated dystrophin gene transfer and upregulation of a natural dystrophin analogue , utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Our results bring strong support to the hypothesis that muscle wasting in dystrophin deficient DMD patients could be prevented by upregulation of utrophin . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We have studied this expression of utrophin immunocytochemically on mature fibres in 42 cases of DMD and BMD , aged 3 months 24 years of age . ^^^ Our data show that the abnormal expression of utrophin on mature muscle fibres in DMD and BMD is not a continuation of the expression that occurs in fetal or regenerating muscle , but is a secondary event caused by unknown factors . ^^^ As all cases studied had some expression of utrophin on mature fibres , this may be a useful additional tool for distinguishing BMD from other dystrophies , especially in cases with minimal abnormalities in dystrophin expression and / or no detectable mutation in the gene . . ^^^ Utrophin is a 395 kDa protein with considerable homology to dystrophin . ^^^ Some revertant fibres , but not all , expressed utrophin and dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| As in DMD , utrophin appears to play an important compensatory role in hamster dystrophinopathy . . ^^^ Reduced sarcolemmal dystrophin distribution and upregulation of utrophin in the cardiac and skeletal muscles of CHF 146 dystrophic hamsters . ^^^ Two other cytoskeletal proteins , spectrin and utrophin , bear remarkable structural and functional homologies to dystrophin . ^^^ Although DH present a suitable model for HMD , there are controversies concerning their dystrophin and utrophin status . ^^^ Using immunocytochemistry and Western blotting , we studied dystrophin , spectrin and utrophin anomalies in the cardiac and skeletal muscles of 6 mo old male DH . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Beginning with postnatal day ( PD ) 1 in both fiber types dystrophin , dystrophin associated glycoproteins ( DAG ) , beta dystroglycan , alpha sarcoglycan ( adhalin ) and spectrin were present in the junctional and extrajunctional sarcolemma , while utrophin , acetylcholinesterase , alpha bungarotoxin labeled acetylcholine receptors were concentrated in the NMJ of both fiber types . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Three dystrophin related proteins ( utrophin , dystrophin related protein 2 ( DRP 2 ) , and dystrobrevin ) have been described . ^^^ Sequence alignments including this second dystrobrevin strongly support the concept that two distinct subfamilies exist within the dystrophin family , one composed of dystrophin , utrophin , and DRP 2 and the other composed of alpha and beta dystrobrevin . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Recently , it was demonstrated in a transgenic mouse model that utrophin could functionally compensate for the lack of dystrophin and alleviate the muscle pathology ( Tinsley , J . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The membrane cytoskeleton interface : the role of dystrophin and utrophin . ^^^ Recent studies with transgenic animals have considerably advanced our knowledge of the roles of dystrophin and utrophin in both muscle and non muscle tissues . ^^^ These and other recent findings are discussed in the context of the structure and function of dystrophin and utrophin at the membrane cytoskeleton interface . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Upregulation of endogenous utrophin might also give a relief in DMD patients , since introduction of truncated utrophin gene considerably improved phenotypic expression of mdx mice , when introduced as a transgene . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is a homologue of dystrophin , the protein whose absence is responsible for Duchenne muscular dystrophy ( DMD ) . ^^^ As a first step toward clarifying if adenovirus ( AV ) mediated utrophin transfer is a possible option to treat DMD , we have constructed an AV expressing utrophin ( AdCMV Utr ) and studied utrophin expression after intramuscular injection of mdx mice , the mouse DMD model . ^^^ These data indicate that AV mediated utrophin transfer is an efficient way of utrophin upregulation in muscle and has the potential of becoming a treatment for DMD . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The cardiac expression of the dystrophin related protein utrophin was increased , and the 43 kDa ( beta dystroglycan ) , 50 kDa ( alpha sarcoglycan ) and 59 kDa ( syntrophin ) dystrophin associated proteins ( DAPs ) were co isolated and present in nearly normal amounts in the membrane . ^^^ However , cardiac dystrophin deficiency and increased utrophin expression were associated with loss of extracellular 156 kDa dystrophin associated glycoprotein ( alpha dystroglycan ) binding to the cardiomyocyte membrane . alpha Dystroglycan is responsible for linkage of the dystrophin complex to the extracellular matrix protein laminin . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin is replaced by utrophin in frog heart ; implications for muscular dystrophy . ^^^ The possibility of using utrophin upregulation as a treatment for dystrophin deficient muscular dystrophies has focused attention on the question of how many of dystrophin ' s various functions can be performed by the closely related protein , utrophin . ^^^ In Xenopus heart , little or no dystrophin was found on Western blots but the dystrophin related protein , utrophin , was abundant . ^^^ This utrophin was shown by immunofluorescence microscopy to be associated with cardiac muscle membranes and its distribution was similar to that of dystrophin in rabbit heart . ^^^ The utrophin distribution pattern in the frog heart was shared by beta dystroglycan , a transmembrane protein responsible for localizing both dystrophin and utrophin at cell membranes . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Mice deficient for both dystrophin and the dystrophin related protein , utrophin , ( Dmd ( mdx ) ; Utrn / mice ) die between 6 and 20 weeks of age suffering from severe muscle weakness with joint contractures , pronounced growth retardation and kyphosis , suggesting that dystrophin and utrophin play complementary roles . ^^^ The phenotypic rescue observed demonstrates that the Dmd ( mdx ) ; Utrn / mice are an ideal model for testing gene delivery protocols for the expression of utrophin or dystrophin in skeletal muscle . ^^^ Skeletal muscle specific expression of a utrophin transgene rescues utrophin dystrophin deficient mice . ^^^ In the absence of full length dystrophin and utrophin , the presence of truncated utrophin also decreases muscle fibre regeneration , relocalizes the dystrophin protein complex to the sarcolemma and re establishes a normal expression pattern of developmental muscle proteins . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Differential expression of dystrophin isoforms and utrophin during dibutyryl cAMP induced morphological differentiation of rat brain astrocytes . ^^^ We have identified isoforms of dystrophin and utrophin , a dystrophin homologue , expressed in astrocytes and examined their expression patterns during dibutyryl cAMP ( dBcAMP ) induced morphological differentiation of astrocytes . ^^^ Immunoblot and immunocytochemical analyses showed that full length type dystrophin ( 427 kDa ) , utrophin ( 395 kDa ) , and Dp 71 ( 75 kDa ) , a small type dystrophin isoform , were coexpressed in cultured nondifferentiated rat brain astrocytes and were found to be located in the cell membrane . ^^^ During morphological differentiation of the astrocytes induced by 1 mM dBcAMP , the amount of Dp 71 markedly increased , whereas that of dystrophin and utrophin decreased . ^^^ Northern blot analyses revealed that dBcAMP regulates the mRNA levels of Dp 71 and dystrophin but not that of utrophin . dBcAMP slightly increased the amount of the beta dystroglycan responsible for anchoring dystrophin isoforms and utrophin to the cell membrane . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is normally present exclusively in synaptic regions of skeletal muscle fibers , although it is expressed extrasynaptically in certain pathological situations , where it has been proposed to compensate for the absence of dystrophin in Duchenne muscular dystrophy patients and mdx mice . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The sparing of extraocular muscle in dystrophinopathy is lost in mice lacking utrophin and dystrophin . ^^^ By immunoblotting , the extraocular muscles of mdx mice exhibited increased levels of a dystrophin analog , dystrophin related protein or utrophin . ^^^ Mice lacking expression of utrophin alone , like the dystrophin deficient mdx mouse , showed no pathological alterations in extraocular muscle . ^^^ However , mice deficient in both utrophin and dystrophin exhibited severe changes in both the accessory and principal extraocular muscles , with the eye muscles affected more adversely than other skeletal muscles . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Perhaps the most surprising result of the past year with regard to synaptogenesis is a negative one mice lacking both dystrophin and utrophin have nearly normal neuromuscular junctions . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| An epitope structure for the C terminal domain of dystrophin and utrophin . ^^^ The muscular dystrophy protein , dystrophin , and the closely related protein , utrophin , are large cytoskeletal proteins which link actin microfilaments to the plasma membrane . ^^^ A panel of 38 monoclonal antibodies ( mAbs ) has been produced against the C terminal domains of dystrophin and utrophin . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Following agrin stimulation , alpha dystrobrevin 1 becomes re localised beneath the cell surface into macroclusters that contain acetylcholine receptors and another dystrophin related protein , utrophin . ^^^ Furthermore , we show that alpha dystrobrevin 1 is associated with both utrophin in C 2 cells and dystrophin in mature skeletal muscle . ^^^ Thus alpha dystrobrevin 1 is a component of two protein complexes in muscle , one with utrophin at the neuromuscular junction and the other with dystrophin at the sarcolemma . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Although utrophin is expressed along the sarcolemma in developing , regenerating and DMD muscles , it nonetheless accumulates at the postsynaptic membrane of the neuromuscular junction in both normal and DMD adult muscle fibres . ^^^ Recent studies using transgenic mouse model systems have directly tested this hypothesis and revealed that upregulation of utrophin throughout dystrophic muscle fibres represents indeed , a viable approach for the treatment of DMD . ^^^ Several years ago , an autosomal homologue to dystrophin , termed utrophin , was identified . ^^^ Due to the high degree of sequence identity between dystrophin and utrophin , it has been previously suggested that utrophin could in fact functionally compensate for the lack of dystrophin . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Consequences of the combined deficiency in dystrophin and utrophin on the mechanical properties and myosin composition of some limb and respiratory muscles of the mouse . ^^^ The mechanical properties and the myosin isoform composition were studied in three isolated muscles ( EDL , soleus , diaphragm ) of mutant mice lacking both dystrophin and utrophin ( dko ) . ^^^ They were compared with the corresponding muscles of the normal and the dystrophin deficient ( mdx ) and the utrophin deficient ( uko ) mice . ^^^ These experiments suggest that lack of both dystrophin and utrophin is very detrimental to the mice and that mechanical properties of the muscles may explain the overall phenotype . ^^^ Moreover these results bring some support to the idea that the expression of utrophin in mdx muscle compensates , to some extent , for the lack of dystrophin . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is a regeneration associated protein transiently present at the sarcolemma of regenerating skeletal muscle fibers in dystrophin deficient hypertrophic feline muscular dystrophy . ^^^ Utrophin , an autosomal homologue of dystrophin , has been suggested as a possible therapeutic replacement of dystrophin in Duchenne or Becker muscular dystrophies ( DMD / BMD ) . ^^^ We have undertaken this study to examine the expression of utrophin in the skeletal muscle of dystrophin deficient cats , a spontaneous animal model for dystrophinopathy . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In contrast , alpha dystrobrevin 1 is more highly restricted to the synapse , like the dystrophin homologue utrophin , and preferentially copurifies with utrophin . ^^^ In yeast two hybrid experiments and coimmunoprecipitation of in vitro translated proteins , alpha dystrobrevin 2 binds dystrophin , whereas alpha dystrobrevin 1 binds both dystrophin and utrophin . alpha Dystrobrevin 2 was lost from the nonsynaptic sarcolemma of dystrophin deficient mdx mice , but was retained on the perisynaptic sarcolemma even in mice lacking both utrophin and dystrophin . ^^^ Thus , the distinct distributions of alpha dystrobrevin 1 and 2 can be partly explained by specific associations with utrophin and dystrophin , but other factors are also involved . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Here , we review our studies on neuromuscular development in mutant mice lacking agrin alpha CGRP , rapsyn , MuSK , dystrophin , dystrobrevin , utrophin , laminin alpha 5 , laminin beta 2 , collagen alpha 3 ( 4 ) , the acetylcholine receptor epsilon subunit , the collagenous tail of acetylcholinesterase , fibroblast growth factor 5 , the neural cell adhesion molecule , and tenascin C . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| All biopsy specimens were routinely studied by a battery of 12 histoenzymatic techniques , and immunohistochemistry was performed for spectrin , three domains of dystrophin and two domains of utrophin . ^^^ Inflammatory myopathies presented abnormal overexpression of utrophin and an abnormal dystrophin immunolabeling pattern . ^^^ This overexpression of utrophin appears to be directly related to the decrease in dystrophin . ^^^ We conclude that the study of utrophin is important for the histological interpretation and differential diagnosis of dystrophin related muscular disorders . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Therefore , up regulation of utrophin by drug therapy is a plausible therapeutic approach in the treatment of DMD . ^^^ These results also have important implications for DMD therapies in which utrophin replacement is achieved by delivery using exogenous vectors . . ^^^ The molecular structure of dystrophin is very similar to that of the related protein utrophin . ^^^ Sarcolemmal localization of a truncated utrophin transgene in the dystrophin deficient mdx mouse significantly improves the dystrophic muscle phenotype . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin immunostaining can be ascribed to dystrophin and / or utrophin as well as the DMD ( Duchenne Muscular Dystrophy ) gene short products Dp 140 and Dp 71 as revealed by Western immunoblots of synaptosomes isolated from neurohypophyses of control rats . ^^^ Dystrophin , utrophin and dystroglycan are present not only in muscle but also in brain . ^^^ Dystrophin immunostaining can be ascribed to dystrophin and / or utrophin as well as the DMD ( Duchenne Muscular Dystrophy ) gene short products Dp 140 and Dp 71 as revealed by Western immunoblots of synaptosomes isolated from neurohypophyses of control rats . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The 2 . 0 A structure of the second calponin homology domain from the actin binding region of the dystrophin homologue utrophin . ^^^ Utrophin is a close homologue of dystrophin , the protein defective in Duchenne muscular dystrophy . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Characterization of dystrophin and utrophin diversity in the mouse . ^^^ Utrophin is a 400 kDa autosomal homolog of dystrophin and a component of the submembranous cytoskeleton . ^^^ While multiple dystrophin isoforms have been identified along with alternatively spliced products , to date only two different mRNA species of utrophin have been identified . ^^^ To determine the degree of evolutionary conservation between dystrophin and utrophin isoforms , we have compared their expression patterns in adult mice . ^^^ Northern blot analysis of multiple adult tissues confirmed that only two major sizes of transcripts are produced from each gene : 13 and 5 . 5 kb from utrophin and 14 and 4 . 8 kb from dystrophin . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Because utrophin can functionally compensate for the lack of dystrophin , the elucidation of the molecular mechanisms regulating utrophin gene transcription may ultimately lead to therapies based on utrophin expression throughout the muscle fibers of Duchenne muscular dystrophy patients . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| MATERIAL AND METHODS : Over three hundred muscle biopsies with a suspected diagnosis of muscular dystrophy were immunostained for dystrophin , dystrophin associated proteins , spectrin and utrophin . ^^^ RESULTS : Duchenne muscular dystrophy showed negative immunostaining and Western blot for dystrophin and dystrophin associated proteins , and overexpression of utrophin . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In order to determine the mechanisms regulating utrophin expression in human skeletal muscle , we examined the expression and distribution of utrophin and its transcript in biopsies from normal subjects as well as from Duchenne muscular dystrophy ( DMD ) and polymyositis ( PM ) patients . ^^^ We first determined by immunoblotting that in comparison to biopsies from normal subjects , utrophin levels were indeed higher in muscle samples from both DMD and PM patients as previously shown . ^^^ By contrast , levels of utrophin mRNAs as determined by both RT PCR assays and in situ hybridization , were identical in muscle samples obtained from normal subjects versus DMD and PM patients . ^^^ The distribution of utrophin transcripts in synaptic and extrasynaptic compartments of muscle fibers obtained from DMD and PM patients was similar to that seen along muscle fibers from normal subjects . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin and utrophin do not play crucial roles in nonmuscle tissues in mice . ^^^ Both Dp 71 , a C terminal dystrophin isoform , and the dystrophin related protein , utrophin , are present at high levels in many nonmuscle tissues . ^^^ To investigate the roles of these proteins in nonmuscle tissues , mice were generated null for utrophin , and deficient in all dystrophin isoforms . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In epithelia , we identified a variation of the dystrophin complex , in which syntrophin , and the dystrophin homologues , utrophin and dystrobrevin , are restricted to the basolateral membrane . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| These include the use of multiple intramuscular injections , increasing the permeability of the extracellular matrix of muscle , inducing mitosis in myoblasts , the use of ex vivo gene transfer , using modified viruses as vectors or synthesized transporter molecules , the use of mechanisms which combat the action of killer T cells , upregulation of isoforms or of alternative proteins such as utrophin for dystrophin and the use of genetic correction methods such as the use of antisense oligonucleotides . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We consider most aspects of mdx skeletal muscle : ( 1 ) the distribution and roles of dystrophin , utrophin , and associated proteins ; ( 2 ) morphological characteristics of the skeletal muscle and hypotheses put forward to explain the regeneration characteristic of the mdx mouse ; ( 3 ) special features of the diaphragm ; ( 4 ) changes in basic fibroblast growth factor , ion flux , innervation , cytoskeleton , adhesive proteins , mastocytes , and metabolism ; and ( 5 ) different lines of therapeutic research . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| METHODS : Examined in the study were the developmental pattern of dystrophins in rat retinae that exhibit inherited progressive photoreceptor degeneration ; dystrophins messengers expression in the outer and the inner retina of normal rats , prepared by mechanical fractionation through the outer plexiform layer ; and immunolocalization of dystrophin proteins and utrophin in normal and degenerated adult rat retinae , with several antibodies prepared against specific regions of the dystrophin superfamily . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin / dystrophin related protein is the autosomal homologue of the chromosome 10 encoded dystrophin protein . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is a close homolog of dystrophin , the protein whose mutations cause Duchenne muscular dystrophy ( DMD ) . ^^^ Utrophin is present at low levels in normal and dystrophic muscle , whereas dystrophin is largely absent in DMD . ^^^ To establish if adenovirus ( AV ) mediated utrophin gene transfer is a possible option for the treatment of DMD , an AV vector expressing a shortened version of utrophin ( AdCMV Utr ) was constructed . ^^^ The effect of utrophin overexpression was investigated following intramuscular injection of this AV into mdx mice , the mouse model of DMD . ^^^ In such cases , the replacement of dystrophin using a utrophin gene transfer strategy could be more advantageous because utrophin would not be a neoantigen . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Up 71 and up 140 , two novel transcripts of utrophin that are homologues of short forms of dystrophin . ^^^ These transcripts appear to be structural homologues of the short dystrophin transcripts , Dp 140 and Dp 71 , emphasizing the high degree of structural conservation between the utrophin and dystrophin genes . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| SAST and syntrophin were highly associated with purified microtubules and microtubule associated proteins , whereas utrophin and dystrophin were only partially associated with microtubules . ^^^ Our data suggest that MAST 205 and SAST link the dystrophin / utrophin network with microtubule filaments via the syntrophins . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| These findings seem to dampen the enthusiasm raised by the prospect of DMD treatment by utrophin rescue in skeletal muscle fibers , as the myocardium would still remain severely affected . . ^^^ Could utrophin rescue the myocardium of patients with dystrophin gene mutations . ^^^ The spontaneous up regulation of utrophin , observed in dystrophin deficient skeletal muscle fibers , may decrease the susceptibility of such fibers to necrosis . ^^^ We studied their explanted heart specimen and / or endoImyocardial biopsies by immunohistochemistry and Western blot for both dystrophin and utrophin . ^^^ Our results suggest that in these patients the up regulation of utrophin in dystrophin deficient cardiomyocytes was unable to prevent the development of life threatening myocardial dysfunction . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Since in skeletal muscle ( 1 ) utrophin is a synaptic homolog to dystrophin , and ( 2 ) the utrophin promoter contains an E box , we examined , in the present study , expression of the utrophin gene during myogenic differentiation using the mouse C 2 muscle cell line . ^^^ Altogether , these results show that in comparison to other synaptic proteins and to dystrophin , expression of the utrophin gene is only moderately increased during myogenic differentiation . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin and dystrophin associated glycoproteins in normal and dystrophin deficient cardiac muscle . ^^^ In this study , various members of the dystrophin family ( dystrophin , the short dystrophin product Dp 71 , utrophin and DRP 2 ) , and different members of the dystrophin associated glycoprotein ( DAG ) complex ( beta dystroglycan , alpha , beta , gamma and delta sarcoglycans ) were localized in bovine cardiac muscle using a battery of specific antibodies . ^^^ In dystrophin deficient cardiac muscle , utrophin and beta sarcoglycan were observed in intercalated disks and at the sarcolemma of each cardiocyte . ^^^ Our results revealed that complexes of associated glycoproteins differ in cardiac muscle when associated with dystrophin or utrophin . ^^^ Despite the described sequence homologies between dystrophin and utrophin , the present results indicate that these proteins have different roles in some specific cardiac cell areas . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin and utrophin complexed with different associated proteins in cardiac Purkinje fibres . ^^^ In mature skeletal muscle , utrophin is a dystrophin related protein localized mainly at the neuromuscular junction , with the same properties as dystrophin in terms of linking the protein complex . ^^^ Utrophin could potentially overcome the absence of dystrophin in dystrophic skeletal muscles . ^^^ In cardiac muscle , dystrophin and utrophin were both found to be present with a distinct subcellular distribution in Purkinje fibres , i . e . utrophin was limited to the cytoplasm , while dystrophin was located in the cytoplasmic membrane . ^^^ In this study , we used this particular characteristic of cardiac Purkinje fibres and demonstrated that associated proteins of dystrophin and utrophin are different in this structure . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We previously reported a patient with Becker muscular dystrophy ( BMD ) who exhibited a benign clinical phenotype and marked expression of utrophin on the muscle cell membrane . ^^^ Although utrophin up regulation in muscle is thought to prevent the muscle wasting in dystrophin deficient DMD or BMD , the data obtained in the present study indicate that up regulated utrophin may have an unexpected influence on the function of the vascular or coagulation system . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The delta sarcoglycan deficient cardiomyopathic hamster and mice deficient in both dystrophin and utrophin showed loss of the smooth muscle sarcoglycan complex , whereas the complex was unaffected in alpha sarcoglycan null mice in agreement with the finding that alpha sarcoglycan is not expressed in smooth muscle cells . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Here we show that the dystrophin deficient mdx mouse and a mouse lacking both dystrophin and the dystrophin related protein , utrophin ( dko ) , have abnormal electrocardiograms ( ECGs ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Mutations in the dystrophin gene ( DMD ) and in genes encoding several dystrophin associated proteins result in Duchenne and other forms of muscular dystrophy . alpha Dystroglycan ( Dg ) binds to laminins in the basement membrane surrounding each myofibre and docks with beta Dg , a transmembrane protein , which in turn interacts with dystrophin or utrophin in the subplasmalemmal cytoskeleton . alpha and beta Dgs are thought to form the functional core of a larger complex of proteins extending from the basement membrane to the intracellular cytoskeleton , which serves as a superstructure necessary for sarcolemmal integrity . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We have previously shown that utrophin , the autosomal homologue of dystrophin , is able to compensate for the absence of dystrophin in a mouse model of DMD ; we have therefore undertaken a detailed study of the transcriptional regulation of utrophin to identify means of effecting its up regulation in DMD muscle . ^^^ These findings significantly contribute to understanding the molecular physiology of utrophin expression and are important because the promoter reported here provides an alternative target for transcriptional activation of utrophin in DMD muscle . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In skeletal muscle , alpha dystrobrevin is a component of the dystrophin associated glycoprotein complex and is localized to the sarcolemma , presumably through interactions with dystrophin and utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The revealed protein band , with 140 kDa molecular weight , was related to dystrophin , utrophin or dystrophin related protein 2 ( DRP 2 ) according to the specificities of the antibodies used to detect them . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin lacks the rod domain actin binding activity of dystrophin . ^^^ Because of the importance of actin binding to the presumed physiological role of dystrophin , we sought to determine whether the autosomal homologue of dystrophin , utrophin , shared this rod domain actin binding activity . ^^^ We therefore produced recombinant proteins representing the cluster of basic repeats of the dystrophin rod domain ( DYSR 11 17 ) or the homologous region of the utrophin rod domain ( UTROR 11 16 ) . ^^^ We present these findings as further support for the electrostatic nature of the interaction of the dystrophin rod domain with F actin and suggest that utrophin interacts with the cytoskeleton in a manner distinct from dystrophin . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is expressed , at the neuromuscular junction , in normal and DMD muscles and there is evidence that it may perform the same cellular functions as dystrophin . ^^^ Nitric oxide and l arginine cause an accumulation of utrophin at the sarcolemma : a possible compensation for dystrophin loss in Duchenne muscular dystrophy . ^^^ An approach to treatment is to compensate for dystrophin loss with utrophin , another cytoskeletal protein with over 80 % homology with dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Alpha dystroglycan ( alpha DG ) is part of a complex of cell surface proteins linked to dystrophin or utrophin , which is distributed over the myofiber surface and is concentrated at neuromuscular junctions . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Expression of Dp 71 in Mller glial cells : a comparison with utrophin and dystrophin associated proteins . ^^^ In morphologically preserved differentiated Mller cells , Dp71f was localized in clusters , utrophin was diffusely distributed in the cytoplasm , and dystrophin associated proteins ( DAPs ) were membrane bound . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| BACKGROUND : Utrophin is a large multidomain protein that belongs to a superfamily of actin binding proteins , which includes dystrophin , alpha actinin , beta spectrin , fimbrin , filamin and plectin . ^^^ Utrophin is the autosomal homologue of dystrophin , the protein defective in the 10 linked Duchenne and Becker muscular dystrophies , and upregulation of utrophin has been suggested as a potential therapy for muscular dystrophy patients . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The utrophin gene codes for a large cytoskeletal protein closely related to dystrophin . ^^^ Because utrophin can functionally substitute dystrophin , the identification and characterization of new regulatory elements provide new targets for possible therapies of Duchenne muscular dystrophy aiming at the up regulation of the utrophin expression in muscle cells . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Our results demonstrate that both the WW and EF hand domains of dystrophin and utrophin , an autosomal homologue of dystrophin , directly bind the cytoplasmic domain of dystroglycan . ^^^ This is the first demonstration of a direct interaction between a dystrobrevin or utrophin and dystroglycan , and has implications for the organization of the dystrophin glycoprotein complex and the use of dystrophin homologues in muscular dystrophy therapy . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin may be a precursor of dystrophin during skeletal muscle development . ^^^ Expression patterns of utrophin were investigated and compared to those of dystrophin and associated proteins in skeletal muscle of rat embryos from E 12 to E 21 by immunohistochemistry . ^^^ Utrophin was readily detected from E 12 on , earlier than full length dystrophin on E 14 . ^^^ The level of utrophin reached a maximum on E 16 17 and then declined while that of dystrophin increased after E 17 . ^^^ Sarcoglycans , appearing from E 14 on , were anchored first by utrophin and then by dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin and utrophin : genetic analyses of their role in skeletal muscle . ^^^ This review summarizes the experiments using transgenic and knockout mouse models that have defined the roles of dystrophin , and the dystrophin related protein utrophin at the skeletal muscle membrane and at the neuromuscular junction . ^^^ Knockouts and transgenics of utrophin have shown this protein to be sufficient to functionally compensate for dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We have previously shown that the dystrophin related protein , utrophin is able to compensate for the lack of dystrophin in the mdx mouse , the mouse model for DMD . ^^^ Prevention of the dystrophic phenotype in dystrophin / utrophin deficient muscle following adenovirus mediated transfer of a utrophin minigene . ^^^ Here , we explore whether utrophin delivered to the limb muscle of dystrophin / utrophin deficient double knockout ( dko ) neonatal mice can protect the muscle from subsequent dystrophic damage . ^^^ Utrophin delivery may avoid the potential problems of an immune response associated with the delivery of dystrophin to a previously dystrophin deficient host . ^^^ These results demonstrate that the utrophin minigene delivered using an adenoviral vector is able to afford protection to the dystrophin / utrophin deficient muscle of the dko mouse . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Because the major DG binding partners in the GBM ( laminin , agrin , perlecan ) , and the intracellular dystrophin analogue utrophin are also present in glomeruli , it appears that podocytes adhere to the GBM via DG complexes , similar to muscle fibers in which actin is linked via dystrophin and DG to the extracellular matrix . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is a large ubiquitously expressed cytoskeletal protein , homologous to dystrophin , the protein disrupted in Duchenne muscular dystrophy . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We also analyzed double and triple mutants lacking other cytoskeletal DGC components ( utrophin and dystrophin ) and myotubes lacking a alpha DB or a transmembrane DGC component ( dystroglycan ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Previous studies reported that dystrophin , utrophin , syntrophin and beta dystroglycan were expressed in the cerebellum . ^^^ Confocal microscopy showed that dystrobrevin was expressed around blood vessels and under the pia mater as dystrophin , utrophin and beta dystroglycan were . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The upregulation of endogenous utrophin in skeletal muscle may lead to a new approach to the treatment of Duchenne muscular dystrophy ( DMD ) . ^^^ Our findings provide an important clue to understanding the mechanism of utrophin expression and the development of an effective treatment for DMD . . ^^^ In normal C57BL / 10 mice , utrophin was also upregulated in adenovirus injected skeletal muscles , where upregulated utrophin often coexisted with dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| However , in contrast to the localization of dystrophin in extrajunctional regions of muscle fibers , utrophin preferentially accumulates at the postsynaptic membrane of the neuromuscular junction in both normal and DMD adult muscle fibers . ^^^ Since it has recently been suggested that the upregulation of utrophin might functionally compensate for the lack of dystrophin in DMD , considerable interest is now directed toward the elucidation of the various regulatory mechanisms presiding over expression of utrophin in normal and dystrophic skeletal muscle fibers . ^^^ Several years ago , an autosomal homologue to dystrophin , termed utrophin , was identified and shown to be expressed in a variety of tissues , including skeletal muscle . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin and utrophin are known to link the intracellular cytoskeleton to the extracellular matrix via a transmembraneous glycoprotein complex . ^^^ Four short C terminal isoforms ( Dp 71 , Dp 116 , Dp 140 , and Dp 260 ) are described for dystrophin and three for utrophin ( Up 71 , Up 113 , and Up 140 ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The carboxy terminal region of utrophin , like the homologous proteins dystrophin , Drp 2 and dystrobrevins , contains structural domains frequently involved in protein protein interaction . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Comparisons with the structures of utrophin and fimbrin ABDs reveal that the conformations of the individual CH domains are very similar to those of dystrophin but that the arrangement of the two CH domains within the ABD is altered . ^^^ The dystrophin dimer reveals a change of 72 degrees in the orientation of one pair of CH 1 and CH 2 domains ( from different monomers ) relative to the other pair when compared with the utrophin dimer . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin and utrophin influence fiber type composition and post synaptic membrane structure . ^^^ To identify potential non mechanical roles of dystrophin , we tested the ability of various truncated dystrophin transgenes to prevent any of the skeletal muscle abnormalities associated with the double knockout mouse deficient for both dystrophin and the dystrophin related protein utrophin . ^^^ We show that restoration of the DAPC with Dp 71 does not prevent the structural abnormalities of the post synaptic membrane or the abnormal oxidative properties of utrophin / dystrophin deficient muscle . ^^^ In marked contrast , a dystrophin protein lacking the cysteine rich domain , which is unable to prevent dystrophy in the mdx mouse , is able to ameliorate these abnormalities in utrophin / dystrophin deficient mice . ^^^ These experiments provide the first direct evidence that in addition to a mechanical role and relocalization of the DAPC , dystrophin and utrophin are able to alter both structural and biochemical properties of skeletal muscle . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Differential expression of utrophin and dystrophin in CNS neurons : an in situ hybridization and immunohistochemical study . ^^^ The cellular distribution of utrophin , the autosomal homologue of dystrophin , was investigated in developing and adult rat and mouse brain by in situ hybridization and immunohistochemistry . ^^^ Double labeling analysis revealed that utrophin and dystrophin are differentially expressed on the cellular and subcellular levels in juvenile and adult brain . ^^^ Likewise , in mice lacking full length dystrophin isoforms ( mdx mice ) , no change in utrophin expression and distribution could be detected in brain , although utrophin was markedly up regulated in muscle cells . ^^^ These results suggest that utrophin and dystrophin are independently regulated and have distinct functional roles in CNS neurons . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Up regulation of utrophin gene expression is recognized as a plausible therapeutic approach in the treatment of Duchenne muscular dystrophy ( DMD ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Syne 1 contains multiple spectrin repeats similar to those found in dystrophin and utrophin , as well as a domain homologous to the carboxyl terminal of Klarsicht , a protein associated with nuclei and required for a subset of nuclear migrations in Drosophila . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Differential expression of dystrophin , utrophin , and dystrophin associated proteins in human muscle culture . ^^^ Desmin immunoreactivity ( IR ) was detected by 3 days in vitro ( DIV 3 ) , IR for developmental heavy chain myosin , vimentin , utrophin , and beta dystroglycan , as well as alpha , beta , and gamma sarcoglycan , a day later . delta Sarcoglycan was found by DIV 7 ; dystrophin could be detected only by DIV 11 . ^^^ This sequence of events during muscle development gives further support to our suggestion that utrophin could be a precursor of dystrophin during development and regeneration . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| DMD gene product , dystrophin , is a submembranous cytoskeletal protein and many dystrophin associated proteins ( DAPs ) have been identified , such as utrophin , dystroglycans , sarcoglycans , syntrophins and dystrobrevins . ^^^ DMD gene product , dystrophin , is a submembranous cytoskeletal protein and many dystrophin associated proteins ( DAPs ) have been identified , such as utrophin , dystroglycans , sarcoglycans , syntrophins and dystrobrevins . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Mller glial cells from rat retina express dystrophin protein Dp 71 , utrophin and the members of the dystrophin associated glycoprotein complex ( DGC ) , namely beta dystroglycan , delta and gamma sarcoglycans and alpha 1 syntrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Neuronal nitric oxide synthase , a component of the dystrophin protein complex , is absent from the sarcolemma of the alpha Syn ( / ) mice , even where other syntrophin isoforms are present . alpha Syn ( / ) neuromuscular junctions have undetectable levels of postsynaptic utrophin and reduced levels of acetylcholine receptor and acetylcholinesterase . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In contrast , a closely related protein called utrophin is not foreign to DMD patients and is able to compensate for dystrophin deficiency when overexpressed throughout development in transgenic mice . ^^^ In this study , dystrophin and utrophin gene transfer effects on dystrophic muscle function were directly compared in the murine ( mdx ) model of DMD using E1 / E3 deleted adenovirus vectors containing either a dystrophin ( AdV Dys ) or a utrophin ( AdV Utr ) transgene . ^^^ Differential effects of dystrophin and utrophin gene transfer in immunocompetent muscular dystrophy ( mdx ) mice . ^^^ In addition , in mature mdx mice , there was significantly greater transgene persistence and reduced inflammation with utrophin compared to dystrophin gene transfer . ^^^ We conclude that dystrophin and utrophin are largely equivalent in their intrinsic abilities to prevent the development of muscle necrosis and weakness when expressed in neonatal mdx animals with an immature immune system . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| An approach to the search for a treatment is to compensate for dystrophin loss by utrophin , another cytoskeletal protein . ^^^ During development , in normal as in dystrophic embryos , utrophin is found at the membrane surface of immature skeletal fibres and is progressively replaced by dystrophin . ^^^ Thus , it is possible to consider utrophin as a ' foetal homologue ' of dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Here we show that in a yeast two hybrid assay its cytoplasmic domain binds beta 2 syntrophin , a modular adapter which in muscle cells interacts with members of the dystrophin family including utrophin , as well as the signaling molecule neuronal nitric oxide synthase ( nNOS ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| These alpha helices , each termed a syntrophin binding motif , are also highly conserved in dystrophin and utrophin . ^^^ Together these data show that there are four potential syntrophin binding sites per dystrophin complex in skeletal muscle : two on dystrobrevin and two on dystrophin or utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The fundamental function of the membrane associated cytoskeletal proteins dystrophin and utrophin remains unclear . ^^^ Given the presence of utrophin in the cholinergic neuromuscular junction , and perturbations of cholinergic transmission in dystrophin deficient nematodes , our findings may suggest a role for DRP 2 in the organization of central cholinergic synapses . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| For most parameters tested , recovery amounted to 80 % , demonstrating that utrophin can very efficiently act as a surrogate for dystrophin . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| These peripheral nerve SGs were colocalized at the outermost layer of the myelin sheath of nerve fibers together with the dystroglycan complex , utrophin , and a short dystrophin isoform ( Dp 116 ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| They were stained immunohistochemically for dystrophin and different markers of differentiation such as desmin , vimentin and utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystroglycan is part of a multimolecular complex , either associated with dystrophin ( the dystrophin associated protein complex ) at the sarcolemma or with utrophin ( the utrophin associated protein complex ) at the neuromuscular junction . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The dystrophin / utrophin homologues in Drosophila and in sea urchin . ^^^ Here we describe the full length product which shows strong structural similarity and sequence identity to human dystrophin and utrophin . ^^^ As in mammals , dmDp 186 and the dmDLPs share the same C terminal and cysteine rich domains which are very similar to the corresponding domains in human dystrophin and utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The utrophin gene codes for a large cytoskeletal protein closely related to dystrophin which , in the absence of dystrophin , can functionally substitute it . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The dystrophic phenotype is associated with elevated levels of creatine kinase activity , Evans blue dye uptake into muscle fibers , reduced amount of dystrophin and upregulation of utrophin expression suggesting a destabilization of the sarcolemma components . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| To test whether the alpha 7 beta 1 integrin can compensate for the absence of dystrophin , we expressed the rat alpha 7 chain in mdx / utr ( / ) mice that lack both dystrophin and utrophin . ^^^ Thus , bolstering alpha 7 beta 1 integrin mediated association of muscle cells with the extracellular matrix alleviates many of the symptoms of disease observed in mdx / utr ( / ) mice and compensates for the absence of the dystrophin and utrophin mediated linkage systems . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| It has been shown that expression or microinjection of amino terminal fragments of dystrophin or the closely related utrophin resulted in the localization of these protein domains to actin bundles . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Alterations in dystrophin and utrophin expression parallel the reorganization of GABAergic synapses in a mouse model of temporal lobe epilepsy . ^^^ Dystrophin and its autosomal homologue utrophin are coexpressed in muscle cells , and utrophin is functionally able to replace dystrophin in models of Duchenne muscular dystrophy . ^^^ Dystrophin is associated with postsynaptic GABA ( A ) receptors in hippocampus , cortex and cerebellum , whereas utrophin is present extrasynaptically , notably in large brainstem neurons . ^^^ Here , the regulation of dystrophin and utrophin was investigated in a model of temporal lobe epilepsy . ^^^ These results suggest that utrophin provides structural support of neuronal membranes , whereas dystrophin is a component of GABAergic synapses . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is the autosomal homologue of dystrophin . ^^^ In Xp 21 muscular dystrophies , utrophin is also detected on the sarcolemma of skeletal and cardiac muscle , while dystrophin is absent or reduced . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In the brain , different neuronal subtypes and glial cells may express dystroglycan in complex with distinct cytoplasmic proteins such as dystrophin , utrophin and their truncated forms . ^^^ Dystroglycan immunostaining was also detected in perivascular astrocytes and in those facing the pia mater , where utrophin and dystrophin truncated isoforms are present . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Structural comparison of actin binding in utrophin and dystrophin . ^^^ It has been proposed that elevating the levels of utrophin , a close homologue of dystrophin , may act as a therapy for these forms of muscular dystrophy . ^^^ In both utrophin and dystrophin , the main actin binding region is at the N terminus . ^^^ There are even differences between utrophin and dystrophin . ^^^ These studies imply that some caution should be applied to claims that utrophin and dystrophin are completely functionally interchangeable . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| These approaches involve the efficient , non antigenic gene transfer for in vivo gene therapy , pharmacological upregulation of the synthesis of utrophin , a related protein that compensates for the loss of dystrophin , and myogenic stem cell transplantation . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In nonneuronal cells , the cell surface protein dystroglycan links the intracellular cytoskeleton ( via dystrophin or utrophin ) to the extracellular matrix ( via laminin , agrin , or perlecan ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| NOS expression in dystrophinopathies can be reduced by somatic gene transfer of dystrophin or utrophin . ^^^ MATERIALS AND METHODS : NADPH d reactivity , iNOS and nNOS ( NOS 1 ) immunolocalization were studied in muscles from mdx mice before and after somatic gene transfer of dystrophin or utrophin . ^^^ The expression of dystrophin and the overexpression of utrophin using adenovirus mediated constructs reduced the number of iNOS positive fibers in mdx quadriceps muscles . ^^^ The correction of some pathology in mdx by dystrophin expression or utrophin overexpression was independent of the presence of nNOS . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Accordingly , these findings provide novel targets , in addition to transcriptional events , for which pharmacological interventions may be envisaged to ultimately increase the endogenous levels of utrophin in skeletal muscle fibers from Duchenne muscular dystrophy ( DMD ) patients . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| These findings have important implications for the feasibility of the up regulation of utrophin in therapy for DMD since they suggest that tissue specific up regulation may not be necessary . . ^^^ We have previously demonstrated that the dystrophin related protein , utrophin is able to compensate for the loss of dystrophin in the mdx mouse , the mouse model of the disease . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| To develop successful treatment of DMD , the authors believe that several different approaches should be used , such as cell transfer therapy , drug design to up regulate utrophin , or a strategy to repair the mutation in vivo . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| This study provides a basis for further understanding the regulatory mechanisms that control utrophin expression in muscle and may facilitate the development of reagents to effect therapeutic up regulation of utrophin in DMD . . ^^^ Utrophin is the autosomal homologue of dystrophin . ^^^ We previously demonstrated that overexpression of utrophin in the muscles of dystrophin null transgenic mice completely prevented the phenotype arising from dystrophin deficiency . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In contrast to dystrophin , which is expressed along the length of healthy muscle fibers , utrophin accumulates at the neuromuscular junction in both normal and DMD fibers . ^^^ The results should therefore , identify specific targets that may become important in designing specific pharmacological interventions directed at increasing the expression of utrophin into extrasynaptic regions of DMD muscle fibers . ^^^ A candidate for such a role is the dystrophin related protein now referred to as utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We have demonstrated an overexpression of utrophin , visualised by immunofluorescence and quantified by Western blotting , in normal myotubes and in mdx ( the animal model of DMD ) myotubes , as in normal ( C 57 ) and mdx mice , both treated with nitric oxide ( NO ) donor or L arginine , the NOS substrate . ^^^ An alternative to the pharmacological approach is to compensate for dystrophin loss by the upregulation of another cytoskeletal protein , utrophin . ^^^ Utrophin and dystrophin are part of a complex of proteins and glycoproteins , which links the basal lamina to the cytoskeleton , thus ensuring the stability of the muscle membrane . ^^^ There is evidence that utrophin may be capable of performing the same cellular functions as dystrophin and may functionally compensate for its lack . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Developmentally regulated expression and localization of dystrophin and utrophin in the human fetal brain . ^^^ Expression of dystrophin and the dystrophin related protein utrophin has been studied in the human fetal brain both in vivo and in vitro . ^^^ Dystrophin localization was similar but not identical to utrophin . ^^^ Results suggest that utrophin and dystrophin are likely to play a key , though different , role in the immature brain . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| A quantitative study of bioenergetics in skeletal muscle lacking utrophin and dystrophin . ^^^ Muscle energetics and function were investigated in the hindlimb of mice lacking dystrophin ( mdx ) , utrophin and dystrophin ( utr dys ) and controls ( C57Bl / 10 ) using 31P and 1H magnetic resonance techniques , electrical nerve stimulation and direct biochemical analysis . ^^^ The data indicate that the severe abnormalities which are present in the absence of utrophin and dystrophin leave basic muscle energetics intact and appear confined to processes involving the sarcolemma . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In particular we describe the potential for differential binding of the b dystroglycan WW domain ligand by dystrophin or caveolin 3 in skeletal muscle and show how this could act as a switch to alter the relative affinity of the muscle dystroglycan complex for caveolin 3 or dystrophin and utrophin . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is an autosomal homolog of the DMD gene product dystrophin , and augmented expression of endogenous utrophin is expected to provide an alternative therapeutic approach to DMD . ^^^ We previously reported that an immune response against a beta galactosidase expressing adenovirus vector , AxCALacZ , resulted in an accumulation of endogenous utrophin on the extrasynaptic sarcolemma in dystrophin deficient mdx mice . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The utrophin gene codes for a large cytoskeletal protein closely related to dystrophin , the gene mutated in Duchenne ' s muscular dystrophy . ^^^ Although utrophin could functionally substitute for dystrophin , in Duchenne ' s muscular dystrophy patients it did not compensate for the absence of dystrophin because in adult muscle utrophin was poorly expressed and limited to subsynaptic nuclei . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystroglycan binds utrophin , a short dystrophin isoform ( Dp 116 ) , and dystroglycan related protein 2 ( DRP 2 ) , all of which are part of a macromolecular complex . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In DMD , the expression of many dystrophin associated proteins ( DAPs ) is reduced along the sarcolemmal membrane , but the same proteins remain concentrated at the neuromuscular junction where utrophin , a dystrophin homologue , is expressed [ Matsumura , K . , Ervasti , J . ^^^ This outcome has led to the concept that ectopic expression of a `` synaptic scaffold ' ' of DAPs and utrophin along myofibers might compensate for the molecular defects in DMD . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunogold confirmation that utrophin is localized to the normal position of dystrophin in dystrophin negative transgenic mouse muscle . ^^^ It has been shown previously that when utrophin is highly expressed in mice which lack dystrophin , the muscle pathology is prevented . ^^^ Immunohistochemical evidence strongly suggests that utrophin in these transgenic mice occupies the position normally filled by dystrophin , although it is not possible to establish this firmly at the level of the light microscope . ^^^ Using the higher resolution provided by the electron microscope , we demonstrate here by immunogold labelling with both monoclonal and polyclonal antibodies that utrophin , in both its truncated and full length forms , is indeed specifically located in the subcellular position usually occupied by dystrophin in normal muscle . ^^^ Furthermore , when both utrophin and dystrophin were labelled in a transgenic line in which both were simultaneously expressed , the sites of both proteins were in the same zone in relation to the plasma membrane . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin , the autosomal homologue of dystrophin , the Duchenne muscular dystrophy gene product , is a cytoskeletal protein found in many tissues . ^^^ Transgenic overexpression of utrophin prevents degeneration of dystrophin deficient muscle fibers . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Three mouse models of muscular dystrophy : the natural history of strength and fatigue in dystrophin , dystrophin / utrophin , and laminin alpha 2 deficient mice . ^^^ Here we characterized disease progression of three mutant mouse strains of muscular dystrophy : mdx mice , which lack dystrophin ; mdx : utrn / mice , which also lack utrophin ; and dy / dy mice , which are deficient in laminin alpha 2 . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Various dystrophin , utrophin and integrin recombinant cDNAs have been shown to prevent the development of muscular dystrophy in transgenic dystrophic ( mdx ) mice . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin is a member of the spectrin superfamily of proteins and is closely related in sequence similarity and functional motifs to three proteins that constitute the dystrophin related protein family , including the autosomal homologue , utrophin . ^^^ An alternative strategy circumventing many problems associated with somatic gene therapies for Duchenne muscular dystrophy has arisen from the demonstration that utrophin can functionally substitute for dystrophin and its over expression in muscles of dystrophin null transgenic mice completely prevents the phenotype arising from dystrophin deficiency . ^^^ This has lead to an interest in the identification and manipulation of important regulatory regions and / or molecules that increase the expression of utrophin and their delivery to dystrophin deficient tissue . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| This paper summarizes the various aspects of functional recovery obtained in dystrophin deficient muscles of the mdx mice where utrophin was overexpressed . ^^^ The quantitative aspect of the replacement of dystrophin by utrophin is discussed . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| A method to induce utrophin up regulation in muscle should therefore be therapeutically useful in DMD . ^^^ These results have important implications for understanding the biology of utrophin and are crucial for future studies aiming to effect its therapeutic up regulation in DMD patients . . ^^^ Utrophin is a paralogue of dystrophin and can functionally replace it in skeletal muscle . ^^^ We have analyzed the expression of A and B utrophin protein and RNA in dystrophin deficient tissues . ^^^ We conclude that ( 1 ) the previously described expression patterns of utrophin represent a composite of A and B utrophin , ( 2 ) A but not B utrophin is up regulated in dystrophin deficient striated muscle , and ( 3 ) this up regulation occurs post transcriptionally with an additional transcriptional component in skeletal muscle . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Up regulation of utrophin was induced by inflammatory response against adenovirus vector mediated gene transfer and this up regulation is one of promising tools for treatment of DMD . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Metabolic profiles of dystrophin and utrophin expression in mouse models of Duchenne muscular dystrophy . ^^^ Using metabolic profiles of diaphragm , models were built describing dystrophin and utrophin , a dystrophin related protein , expression . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Previously , we have shown in a transgenic mouse model of the disease ( mdx ) that high levels of expression of the dystrophin related protein , utrophin can prevent pathology . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin can functionally replace dystrophin in dystrophin deficient muscle and may have a role in a therapeutic strategy for Duchenne muscular dystrophy . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Pharmacological upregulation of utrophin , the autosomal homologue of dystrophin , offers a potential therapeutic approach to treat Duchenne patients . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Moreover , utrophin , a recently identified structural homologue of dystrophin is reported to be up regulated in DMD . ^^^ In order to investigate the association between utrophin and muscle regeneration in DMD , an immunohistochemical study using antibodies to utrophin , dystrophin , vimentin and desmin was carried out in 17 cases of DMD , 3 cases of polymyositis and 1 case of dermatomyositis . ^^^ Utrophin was positive in 94 . 0 % of DMD and in 75 . 0 % of IM . 36 . 4 % of the myofibers were positive in DMD , as compared to 10 . 5 % in IM ( p=0 . 001 ) . ^^^ These results show that utrophin up regulation is regeneration associated , and that it is proportional to the quantity of regenerating myofibers , but is not specific for DMD . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Duchenne muscular dystrophy is caused by dystrophin deficiency , which can be prevented in the mdx mouse model by over expression of an autosomal homologue , utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The spectrin superfamily ( spectrin , alpha actinin , utrophin and dystrophin ) has in common a triple helical repeating unit of ~106 amino acid residues . ^^^ A profile hidden Markov model of beta spectrin repeat 1 detects alpha actinins , but not utrophin or dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We used monoclonal and polyclonal antibodies produced in our laboratory , which are directed against dystrophin , utrophin , short dystrophin products , alpha dystrobrevin , beta dystroglycan , alpha syntrophin , alpha , beta , gamma , delta , epsilon sarcoglycan , and sarcospan . ^^^ Regardless of the tissue analyzed , at least one dystrophin or utrophin as full length molecule and one short dystrophin product or dystrobrevin as proteins belonging to the dystrophin superfamily were found . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| For this reason , utrophin transfer to dystrophin deficient muscle appears as a promising therapeutic approach to DMD . . ^^^ Utrophin is highly homologous and structurally similar to dystrophin , and in gene delivery experiments in mdx mice was able to functionally replace dystrophin . ^^^ We found reduced fibrosis and increased expression of dystrophin associated proteins ( DAPs ) in association with muscle areas expressing the utrophin minigene , indicating that mini utrophin can functionally compensate for lack of dystrophin in injected muscles . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| An atomic model for actin binding by the CH domains and spectrin repeat modules of utrophin and dystrophin . ^^^ Utrophin and dystrophin link cytoskeletal F actin filaments to the plasmalemma . ^^^ Genetic strategies to replace defective dystrophin with utrophin in individuals with muscular dystrophy requires full characterization of these proteins . ^^^ Here , electron microscopy and 3D reconstruction of F actin decorated with utrophin and dystrophin actin binding constructs were performed using Utr 261 ( utrophin ' s CH domain pair ) , Utr 416 ( utrophin ' s CH domains and first spectrin repeat ) and Dys 246 ( dystrophin ' s CH domain pair ) . ^^^ The CH domain actin interaction for dystrophin was found to be more complex than for utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Here , we will review present pharmacological strategies , in particular those dealing with functional substitution of dystrophin by utrophin and enhancing muscle progenitor commitment by myostatin blockade , with a view toward facilitating drug discovery for DMD . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Analysis of DMD gene products , utrophin and dystrophin associated proteins ( DAPs ) , showed that Dp 71 and utrophin were localized around the blood vessels , in the ganglion cell layer ( GCL ) , and the inner limiting membrane ( ILM ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In addition to these well defined viral strategies , plasmid vectors and the upregulation of utrophin ( a dystrophin homologue ) have potential . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Beta dystroglycan , via its cytoplasmic tail , interacts with dystrophin and utrophin and also with the actin cytoskeleton . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Compensation for dystrophin deficiency : ADAM 12 overexpression in skeletal muscle results in increased alpha 7 integrin , utrophin and associated glycoproteins . ^^^ Indeed , by immunostaining and immunoblotting we found an approximately 2 fold increase in expression , and distinct extrasynaptic localization , of alpha 7B integrin and utrophin , the functional homolog of dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| OBJECTIVE : With a view to further experimental studies on Duchenne muscular dystrophy ( DMD ) , we investigated the motor function , serum creatine kinase ( CK ) level and pathological characteristics of muscular tissue in C 57 , mdx , dystrophin / utrophin gene double knockout ( dko ) mice . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The adverse effects of Dp 71 in muscle are probably due to its competition with dystrophin and utrophin ( in MDX muscle ) for binding to the DAP complex . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Astrocytes were not affected by treatment , as shown by utrophin expression , a dystrophin like protein that was not modified in pure astrocytic cultures . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is a paralogue of dystrophin and can functionally replace it in skeletal muscle . ^^^ A utrophin is up regulated in dystrophin deficient skeletal muscle and we sought to test the hypothesis that this up regulation occurs as a consequence of ongoing regeneration . ^^^ Skeletal muscle in these tissues is dystrophin deficient but not regenerating ; we found that A utrophin up regulation still occurred . ^^^ An alternative hypothesis involving competition for binding sites between utrophin and dystrophin is discussed . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Here we present immunocytochemical localization of beta dystroglycan , the central member of the DGC , utrophin and Dp71f , the spliced 71 kDa dystrophin protein product of the DMD gene , in cultured retinal Muller glial cells . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Two other dystrophin related proteins utrophin and an 87 kDa postsynaptic protein are encoded by separate loci and , like dystrophin , they are components of the neuromuscular junction . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin and dystrophin remained restricted to the endfoot membrane in those cells in which AQP 4 had been redistributed , whereas alpha syntrophin redistributed together with AQP 4 across the entire cell surface . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| ZZ domain is essentially required for the physiological binding of dystrophin and utrophin to beta dystroglycan . ^^^ The binding mode of utrophin is different from that of dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Nitric oxide and cGMP regulate the activity of several kinases , some of which may influence interaction of dystrophin and utrophin with the actin cytoskeleton to mediate or modulate postsynaptic differentiation in muscle cells . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystroglycan is an important cell adhesion receptor linking the actin cytoskeleton , via utrophin and dystrophin , to laminin in the extracellular matrix . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| On chromosome 6 is located the gene of a protein presenting 80 % homology with dystrophin : utrophin , which is expressed at the neuromuscular junction . ^^^ The review examines if utrophin can replace dystrophin and correct the structural and functional characteristics of the myopathy , and how the improvements can be quantitatively expressed . ^^^ In transgenic mice , deficient in dystrophin , but overexpressing large quantities of utrophin , the latter is found on structures where dystrophin is normally located , histological signs of necrosis disappear and the recovery of functional disorders , specially affecting the mechanical properties of the muscle fibres , can be complete . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In fact , pleABD / 2alpha has been found to have the same compact fold as the human plectin ABD and the fimbrin ABD , differing from the open conformation described for the ABDs of utrophin and dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In this investigation , we test whether expression of a nNOS transgene in wild type or mdx muscle increases expression of DPC proteins , or functionally related proteins in the integrin complex that are upregulated in dystrophin deficiency , or affects expression of the dystrophin homolog , utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Muscles of very young and mature normal , mdx , and immunodeficient mice were injected with plasmids expressing beta galactosidase , microdystrophin , full length dystrophin , or full length utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin can function in muscle as a substitute for dystrophin and its over expression has been used successfully to ameliorate mdx muscle pathology . ^^^ Despite of this fact , there are no detailed studies on the expression of endogenous skeletal muscle utrophin and dystrophin associated glycoproteins throughout the life span of mdx mice . ^^^ A lineal correlation between utrophin and beta dystroglycan levels , not seen in controls , indicates that improvement of mdx is due to utrophin binding to dystrophin associated glycoproteins . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The prophylactic effect of deflazacort treatment was associated with increased expression of NF ATc 1 target genes such as the dystrophin homologue utrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| These findings demonstrate the value of including antibodies to nNOS in routine immunohistochemical studies and that absence of nNOS can be a more sensitive marker than up regulation of utrophin for diagnosis of BMD . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Hearts from both models accumulated desmin and beta tubulin , mdx mouse hearts accumulated utrophin and MLP , and MLP null mouse hearts accumulated dystrophin and syncoilin . ^^^ Although the increase in MLP and utrophin in the mdx mouse heart was able to compensate for the loss of dystrophin , accumulation of desmin , syncoilin and dystrophin were unable to compensate for the loss of MLP , resulting in heart failure . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The role of utrophin and Dp 71 for assembly of different dystrophin associated protein complexes ( DPCs ) in the choroid plexus and microvasculature of the brain . ^^^ In the brain , utrophin is present in the choroid plexus epithelium and vascular endothelial cells , whereas the short C terminal isoform of dystrophin ( Dp 71 ) is localized in the glial end feet surrounding blood vessels . ^^^ We investigated the composition and localization of the DPC in dependence on the anchoring proteins in mice lacking either utrophin ( utrophin0 / 0 ) or dystrophin isoforms ( mdx3Cv ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The utrophin gene has two promoters , A and B , and promoter A of the utrophin gene is a possible target of pharmacological interventions for DMD because A utrophin is up regulated in dystrophin deficient mdx skeletal and cardiac muscles . ^^^ Utrophin is an autosomal homologue of dystrophin , and overexpression of the protein is expected to compensate for the defect of dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Here we consider these approaches in terms of three potential goals : improvement of dystrophic phenotype , expression of dystrophin , and overexpression of utrophin . ^^^ Utrophin exhibits 80 % homology with dystrophin and is able to perform similar functions . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| The identification of CAPON transcripts and protein in mammalian muscle and responses to L arginine suggest CAPON may have a functional role in stabilizing neuronal NOS in skeletal muscle in the cytoskeletal complex associated with dystrophin / utrophin , with possible applications to therapy for human muscular dystrophy . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Molecular heterogeneity of the dystrophin associated protein complex in the mouse kidney nephron : differential alterations in the absence of utrophin and dystrophin . ^^^ The dystrophin associated protein complex ( DPC ) consisting of syntrophin , dystrobrevin , and dystroglycan isoforms is associated either with dystrophin or its homolog utrophin . ^^^ Using high resolution immunofluorescence imaging and Western blotting , we have investigated the effects of utrophin and dystrophin gene deletion on the formation and membrane anchoring of the DPC in kidney epithelial cells , which co express utrophin and low levels of the C terminal dystrophin isoform Dp 71 . ^^^ This hypothesis is confirmed by the almost complete loss of all DPC proteins examined in mice lacking full length utrophin and all C terminal dystrophin isoforms ( utrophin ( 0 / 0 ) / mdx ( 3Cv ) ) . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| However , colocalization of SGs and SSPN with dystrophin and utrophin was noted . ^^^ These results , interestingly , suggest that the SG SSPN complex may either form with dystrophin or utrophin in smooth muscle cells , and with utrophin in endothelial cells . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In this context , we review our current knowledge of the mechanisms regulating expression of utrophin , the autosomal homologue of dystrophin . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We report in the present study that the active form of RhoA increases the expression of utrophin , the autosomal homologue of dystrophin in the mouse C2C12 and rat L 8 myoblastic cell lines . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin deficient ( mdx ) mice have a much milder phenotype , whereas double knockout ( DKO ) mice lacking both dystrophin and its homolog , utrophin , exhibit the clinical signs observed in DMD patients . ^^^ Utrophin deficiency worsens cardiac contractile dysfunction present in dystrophin deficient mdx mice . ^^^ Dystrophin deficient ( mdx ) mice have a much milder phenotype , whereas double knockout ( DKO ) mice lacking both dystrophin and its homolog , utrophin , exhibit the clinical signs observed in DMD patients . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| One of the chosen strategies consists of the overexpression of utrophin , a protein 80 % homologous with dystrophin , and able to perform similar functions . ^^^ In this review , we shall consider studies of pharmacological therapy , the aims of which can be classified in three categories : reversal of dystrophic phenotype , dystrophin expression , utrophin overexpression . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| A possible treatment for Duchenne muscular dystrophies would be to compensate for dystrophin loss by increasing the expression of utrophin , another cytoskeletal protein of the muscle membrane . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Utrophin is an autosomal homologue of dystrophin , abnormal expression of which is responsible for 10 linked Duchenne and Becker muscular dystrophy . ^^^ When dystrophin is absent utrophin is abundant on the sarcolemma . ^^^ We also show that muscle adjacent to some soft tissue tumours shows increased sarcolemmal utrophin A , showing that utrophin and dystrophin can simultaneously localise to the sarcolemma and raising the possibility that factor ( s ) from the tumour cells or accompanying inflammatory cells may have a role in regulating utrophin . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| All the 30 muscle biopsies of patients with Duchenne muscular dystrophy ( DMD ) showed all or majority of muscle fibers deficient for dystrophin and positive for utrophin . ^^^ In the 4 female DMD carriers there was mosaic pattern of staining for dystrophin and reciprocal positivity for utrophin . ^^^ This study shows that dystrophin staining differentiates DMD and DMD carriers from other childhood muscular dystrophies and utrophin staining is of no added value . ^^^ Utility of dystrophin and utrophin staining in childhood muscular dystrophy . ^^^ To determine the utility of dystrophin and utrophin staining in the differential diagnosis of childhood muscular dystrophy . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Western blotting was used to measure levels of beta myosin heavy chain ( beta MyHC ) , a marker of hypertrophy , and levels of dystrophin , desmin , MLP , beta tubulin , utrophin and syncoilin , using GAPDH for normalization . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| A promising pharmacological treatment for DMD aims to increase levels of utrophin , a homolog of dystrophin , in muscle fibers of affected patients to compensate for the absence of dystrophin . ^^^ Here , we review recent developments in our understanding of the regulatory pathways that govern utrophin expression , and highlight studies that have used activators of these pathways to alleviate the dystrophic symptoms in DMD animal models . ^^^ The results of these preclinical studies are promising and bring us closer to implementing appropriate utrophin based drug therapies for DMD patients . . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In addition , beta dystroglycan , the transmembrane glycoprotein that anchors the cytoplasmic C terminal domain of utrophin , showed similar increase expression in mdx diaphragm , as opposed to other components of the dystrophin associated protein complex ( DAPC ) such as alpha dystrobrevin 1 and alpha sarcoglycan . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin and utrophin bind actin through distinct modes of contact . ^^^ This study was designed to define the molecular epitopes of dystrophin actin interaction and to directly compare the actin binding properties of dystrophin and utrophin . ^^^ According to our data , dystrophin and utrophin both bound alongside actin filaments with submicromolar affinities . ^^^ Dystrophin and utrophin both stabilized preformed actin filaments from forced depolymerization with similar efficacies but did not appear to compete for binding sites on actin . ^^^ We also found that dystrophin binding to F actin was markedly sensitive to increasing ionic strength , although utrophin binding was unaffected . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In contrast , double mutant mice ( mdx / utrn ( / ) ) , deficient for both dystrophin and utrophin die by approximately 3 months and suffer from severe muscle weakness , growth retardation , and severe spinal curvature . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Transgenic overexpression of the dystrophin homologue utrophin , or functional dystrophin constructs in mdx muscle , restored gamma actin to normal levels , whereas gamma actin remained elevated in mdx muscle expressing nonfunctional dystrophin constructs . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We investigated in diagnostic muscle biopsies from 16 patients with Duchenne muscular dystrophy ( DMD ) the level of utrophin expression using quantitative immunoblot analysis . ^^^ We found that utrophin expression increases with age in DMD and that there is a significant positive correlation between the quantity of utrophin at initial biopsy and time to becoming wheelchair bound . . ^^^ Although there is good experimental data that utrophin , the autosomal analog of dystrophin , can ameliorate the phenotype in dystrophinopathies , there is scant evidence from human data to support this hypothesis . ^^^ |
|
| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Cleavage of utrophin by calpain 1 or 2 generates specific degradation products that are also found in cultured control and DMD myotubes under conditions with elevated intracellular Ca2+ levels . ^^^ These observations suggest that , beside its known effect on general muscle protein degradation , calpain contributes to DMD pathology by specifically degrading the compensatory protein utrophin . . ^^^ Utrophin is a functional homolog of dystrophin that partially compensates for dystrophin deficiency in myofibers of mdx mice . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Over expression of utrophin , the autosomal paralogue of dystrophin , as a transgene in the mdx mouse ( the mouse model of DMD ) has demonstrated that utrophin can prevent the muscle pathology . ^^^ Thus , up regulation of utrophin in DMD muscle is a potential therapy for DMD . ^^^ These results are very encouraging and suggest that pharmacological up regulation of utrophin may well be a feasible approach to therapy for DMD . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| This hypothesis is based on the observation that utrophin , which has 80 % homology with dystrophin , is overexpressing in the dystrophin deficient myofibers . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Role of dystrophin and utrophin for assembly and function of the dystrophin glycoprotein complex in non muscle tissue . ^^^ The dystrophin glycoprotein complex ( DGC ) is a multimeric protein assembly associated with either the 10 linked cytoskeletal protein dystrophin or its autosomal homologue utrophin . ^^^ Here , we focus on recent studies of the DGC in brain , blood brain barrier and choroid plexus , retina , and kidney and discuss the role of dystrophin isoforms and utrophin for assembly of the complex in these tissues . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| In the peripheral nervous system , utrophin and the short dystrophin isoform ( Dp 116 ) are co localized at the outermost layer of the myelin sheath of nerve fibers ; together with the dystroglycan complex . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Interactions of intermediate filament protein synemin with dystrophin and utrophin . ^^^ We show herein that tissue purified avian synemin directly interacts with both dystrophin and utrophin , and that specific expressed regions of both of the mammalian ( human ) synemin isoforms ( alpha synemin and beta synemin ) directly interact with specific expressed domains / regions of the dystrophin and utrophin molecules . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Mice carrying mutations in both the dystrophin and utrophin genes die prematurely as a consequence of severe muscular dystrophy . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunoreactivity of skate electrocytes towards monoclonal antibodies against human dystrophin and dystrophin related ( DMDL ) protein . ^^^ Monoclonal antibodies against a human autosomal homologue of dystrophin ( DMDL protein ) did not detect a similar protein in skate or Torpedo . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| Localization of the DMDL gene encoded dystrophin related protein using a panel of nineteen monoclonal antibodies : presence at neuromuscular junctions , in the sarcolemma of dystrophic skeletal muscle , in vascular and other smooth muscles , and in proliferating brain cell lines . mAbs have been raised against different epitopes on the protein product of the DMDL gene , which is an autosomal homologue of the 10 linked DMD gene for dystrophin . ^^^ The DMDL protein was undetectable in the nonjunctional sarcolemma of normal human muscle , but was observed in nonjunctional sarcolemma of Duchenne muscular dystrophy patients , where dystrophin itself is absent or greatly reduced . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| We have previously reported a dystrophin related locus ( DMDL for Duchenne muscular dystrophy like ) on human chromosome 6 that maps close to the dy mutation on mouse chromosome 10 . ^^^ Unlike the dystrophin gene , the DMDL gene transcript is not differentially spliced at the 3 ' end in either fetal muscle or brain . . ^^^ |
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| Interacting proteins: P46939 and P11532 |
Pubmed |
SVM Score :0.0 |
| To assess the role of mechanical injury in the onset of DMD , extensor digitorum longus ( EDL ) muscles from C 57 ( control ) , mdx , and mdx utrophin deficient [ mdx : utrn ( / ) ; dystrophic ] pups aged 9 12 days were subjected to an acute stretch injury or no stretch protocol in vitro . ^^^ |
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