Pubmed abstracts for Protein-Protein Interaction search result :


Interacting proteins: Q14118 and P11532 Pubmed SVM Score :1.392528
Dystrophin binds with dystroglycan complex which transverse through sarcolemma and then binds with laminin in the basal lamina , forming a long axis between action threads and the extracellular matrix . 1.392528^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.7027089
In muscle beta dystroglycan interacts with dystrophin on the inside of the cell and with alpha dystroglycan , which binds the extracellular matrix protein laminin , on the outside . 0.7027089^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.60055801
By double immunostaining and immunoblotting we show here that beta dystroglycan is expressed in the human retina where it co localizes with dystrophin in photoreceptor synaptic complexes most likely on the postsynaptic side . 0.60055801^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.52241511
Thus , the present results also support the hypothesis that dystrophin interacts with dystroglycan in cerebellar Purkinje neurons . . 0.52241511^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.50701995
Beta dystroglycan , via its cytoplasmic tail , interacts with dystrophin and utrophin and also with the actin cytoskeleton . 0.50701995^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.50014818
The alpha subunit of dystroglycan , a member of the dystrophin associated protein complex , binds to extracellular laminin alpha 2 , while its beta subunit interacts with cytoskeletal dystrophin . 0.50014818^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
In skeletal muscle , merosin is a native ligand for dystroglycan linking the extracellular matrix and dystrophin . ^^^
Interacting proteins: Q14118 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 . ^^^ Because dystroglycan binds laminin , and dystrophin binds actin , the complex containing these two proteins is thought to link the extracellular matrix to the cytoskeleton . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
This suggests that the link between the membrane cytoskeleton and extracellular matrix mediated by dystrophin dystroglycan may play a functional role in brain . ^^^ Further , in situ hybridisation to mouse brain sections shows that the dystroglycan gene is expressed in relatively few structures and co localises with dystrophin mRNA in hippocampus , dentate gyrus , olfactory bulb and Purkinje neurons but , surprisingly , not in the cortex . ^^^ Dystroglycan is also expressed in those brain areas where the dystrophin related protein ( utrophin ) is present . ^^^ Our results provide a basis for a future characterisation of the role of dystrophin dystroglycan association in the brain . . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystrophin associated glycoprotein complex is classified into two subcomplexes : the dystroglycan complex ( 156DAG and 43DAG ) and the sarcoglycan complex ( 50DAG , A3b , and 35DAG ) . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystrophin , the protein product of the Duchenne muscular dystrophy ( DMD ) gene , is associated with a large oligomeric complex of sarcolemmal glycoproteins , including dystroglycan which provides a linkage to the extracellular matrix component , laminin . ^^^ Dystrophin , the protein product of the Duchenne muscular dystrophy ( DMD ) gene , is associated with a large oligomeric complex of sarcolemmal glycoproteins , including dystroglycan which provides a linkage to the extracellular matrix component , laminin . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The 156 kD dystrophin associated glycoprotein ( 156 kD dystroglycan ) specifically bound laminin in a calcium dependent manner and was inhibited by NaCl ( IC 50 = 250 mM ) but was not affected by 1 , 000 fold ( wt / wt ) excesses of lactose , IKVAV , or YIGSR peptides . ^^^ In addition , laminin Sepharose quantitatively precipitated purified dystrophin glycoprotein complex , demonstrating that the laminin binding site is accessible when dystroglycan is associated with the complex . ^^^ However , the other proteins of the striated muscle dystrophin glycoprotein complex appear to be absent , antigenically dissimilar or less tightly associated with dystroglycan in nonmuscle tissues . ^^^ Furthermore , our results suggest that dystrophin and dystroglycan may play substantially different functional roles in nonmuscle tissues . . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystrophin , the protein product of the Duchenne muscular dystrophy ( DMD ) gene , is a cytoskeletal protein tightly associated with a large oligomeric complex of sarcolemmal glycoproteins including dystroglycan , which provides a linkage to the extracellular matrix component , laminin . ^^^ Dystrophin , the protein product of the Duchenne muscular dystrophy ( DMD ) gene , is a cytoskeletal protein tightly associated with a large oligomeric complex of sarcolemmal glycoproteins including dystroglycan , which provides a linkage to the extracellular matrix component , laminin . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystrophin is associated with a large oligomeric complex of sarcolemmal glycoproteins , including the novel laminin binding glycoprotein called dystroglycan , which provides a linkage to the extracellular matrix . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
These results are consistent with previous findings demonstrating that the extracellularly exposed 156 DAG ( dystroglycan ) of the skeletal muscle dystrophin glycoprotein complex binds laminin , a component of the basement membrane . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystrophin is associated with a large oligomeric complex of sarcolemmal glycoproteins , including dystroglycan which provides a linkage to the extracellular matrix component , laminin . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Other dystrophin associated proteins are well preserved in myopathic hamster skeletal muscle , but the link between dystrophin and dystroglycan is disrupted . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystrophin associated proteins ( DAPs ) are classified into a few groups , namely , those comprising of dystroglycan complex , sarcoglycan complex , syntrophin complex and others . ^^^ Subsarcolemmal actin filaments are connected to laminin in the basement membrane through dystrophin and the dystroglycan complex . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystroglycan expression in the wild type and mdx mouse neural retina : synaptic colocalization with dystrophin , dystrophin related protein but not laminin . ^^^ Alpha and beta dystroglycan ( alpha and beta DG ) are members of a dystrophin associated glycoprotein complex ( DGC ) in skeletal muscle which binds to agrin and laminin , and has been postulated to be involved in myoneural snyapse formation . ^^^ Western blot analysis revealed the expression of several dystrophin isoforms in wild type and mdx retina , possibly explaining the unaltered expression of alpha and beta dystroglycan in the mdx central nervous system ( CNS ) . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Alpha dystroglycan ( 156 kDa DAG ) and beta dystroglycan ( 43 kDa DAG ) are encoded by the same gene and are components of the dystrophin associated membrane glycoprotein complex . ^^^ The expression patterns of dystroglycan and dystrophin overlap in major muscle systems during development , suggesting that the dystrophin dystroglycan complex plays an important role during myogenesis . ^^^ In mdx embryos the pattern of distribution of dystroglycan mRNA remains unchanged , as do those of utrophin and apo dystrophin mRNAs . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
This observation suggests that protein phosphorylation may have some role in modulating the function of dystrophin or its interaction with membrane associate dystroglycan . ^^^
Interacting proteins: Q14118 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: Q14118 and P11532 Pubmed SVM Score :0.0
Immunocytochemical analysis using antibodies to merosin , dystrophin , 43 kDa dystroglycan , adhalin , and laminin was normal in 11 of 12 patients . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
In the muscle membrane , dystrophin associates with several proteins and glycoproteins organized in two main subcomplexes : the dystroglycan ( DG ) and sarcoglycan ( SG ) complexes . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Although it is well established that dystroglycan , a transmembrane protein , plays an important role in muscle tissues by bridging intracellular dystrophin to the laminin in the extracellular matrix , its role in non muscle tissues remains elusive . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystroglycan is a cell surface complex which in muscle links the extracellular matrix protein laminin 2 to the membrane associated cytoskeletal protein dystrophin . ^^^ Both isoforms share the dystroglycan binding region of dystrophin but lack the region known to bind to actin . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystroglycan was originally identified as the extracellular and transmembrane constituents of a large oligomeric complex of sarcolemmal proteins associated with dystrophin , the protein product of the Duchenne muscular dystrophy ( DMD ) gene . ^^^ The fact that the drastic reduction of dystroglycan in the sarcolemma , caused by the absence of dystrophin , leads to muscle cell death in DMD patients and mdx mice indicates that , as a laminin receptor , dystroglycan contributes to sarcolemmal stabilization during contraction and stretch of striated muscle cells . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The NH 2 terminus attaches to the cytoskeleton , while the COOH terminus attaches to the dystrophin associated protein ( DAP ) complex , which can be separated into the dystroglycan , sarcoglycan , and syntrophin subcomplexes . ^^^ While the function of each DAP is not known , the dystroglycan complex binds laminin in the extracellular matrix , and binds the dystrophin COOH terminus in vitro . ^^^ Mice lacking this latter domain express very low levels of the sarcoglycans , indicating that the sarcoglycan complex binds dystrophin via dystroglycan . ^^^
Interacting proteins: Q14118 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 . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Deficiency of syntrophin , dystroglycan , and merosin in a female infant with a congenital muscular dystrophy phenotype lacking cysteine rich and C terminal domains of dystrophin . ^^^ However , the expression of dystroglycan and syntrophin was also greatly reduced , and the immunoreactivity for the antibodies against the cysteine rich / C terminal domains of dystrophin was absent in the sarcolemma . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Localization of dystrophin and beta dystroglycan in bovine retinal photoreceptor processes extending into the postsynaptic dendritic complex . ^^^ Dystrophin is an actin binding protein of the membrane cytoskeleton that binds to dystroglycan , an integral membrane protein of the plasma membrane that is posttranslationally cleaved into a transmembrane dystrophin binding beta moiety and an extracellular laminin and agrin binding alpha moiety . ^^^ Here we show by confocal laser microscopy and immunogold electron microscopy that dystrophin and beta dystroglycan are colocalized in bovine rod photoreceptor synaptic complexes distal from the ribbon containing active synaptic zones . ^^^ We speculate that the dystrophin dystroglycan complex of the cavital plasma membrane stabilizes the elaborate synaptic morphology or plays a role in the immobilization of still unknown transporters and receptors involved in certain aspects of neurotransmission to bipolar cells . ^^^ A further outcome of this study is that dystrophin and dystroglycan are located along the vitread membrane surface of Mller cell endfeet where this protein complex may be important for the attachment of the retina to the basal lamina and the vitreous . . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
In view of the abnormal neurotransmission between photoreceptors and ON bipolar cells in DMD patients the dystrophin / beta dystroglycan containing projections of photoreceptor presynaptic terminals into the postsynaptic dendritic plexus might somehow modify the ON bipolar pathway . ^^^ The carboxy terminus of dystrophin binds to a plasma membrane anchor , beta dystroglycan , which is associated on the external side with the extracellular matrix receptor , alpha dystroglycan , that binds to the basal lamina proteins laminin 1 , laminin 2 , and agrin . ^^^ At this location dystrophin colocalizes with dystroglycan . ^^^ Dystrophin and dystroglycan are located distal from the ribbon containing active synaptic zones where both proteins are restricted to the photoreceptor plasma membrane bordering on the lateral sides of the synaptic invagination . ^^^ In addition , some neuronal profiles of the postsynaptic complex also contain dystrophin and beta dystroglycan . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystrophin and dystroglycan messenger RNAs are expressed in specific brain areas , including regions of the cortex and the hippocampus , and in such neurons dystrophin has been localized to postsynaptic densities . ^^^ In the present study we examined by in situ hybridization the effect of neuronal activation and neurotoxicity induced by kainate and pentylenetetrazole administered in vivo on dystrophin and dystroglycan expression in the rat brain . ^^^ For comparison , seizures which are not associated with progressive neurodegeneration were induced by pentylenetetrazole : in this situation the dystrophin and dystroglycan messenger RNA levels remained unchanged in all areas of the hippocampal formation . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The surface component beta dystroglycan is a member of the dystrophin glycoprotein complex providing a trans sarcolemmal linkage between the actin membrane cytoskeleton and the extracellular matrix component laminin alpha 2 . ^^^ We therefore investigated the oligomerization of beta dystroglycan and its connection with the most abundant dystrophin homologues in these two tissues . ^^^ Within these crosslinkable complexes , immuno reactive overlaps were observed between beta dystroglycan , alpha dystroglycan , laminin and 427 kDa dystrophin in diaphragm and skeletal muscle . ^^^ In synaptosomes , the major brain dystrophin isoform Dp 116 also exhibited an immuno reactive overlap with members of the dystroglycan complex . ^^^ These findings demonstrate that beta dystroglycan does not exist as a monomer in native membranes and imply that certain dystrophin isoforms and dystrophin associated components interact with this surface protein in diaphragm and brain as has been previously shown for skeletal and heart muscle . . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
To reduce ascertainment bias , biopsies with dystrophic ( n=131 ) and non dystrophic myopathic ( n=71 ) changes were studied with antibodies to dystrophin , alpha , beta , and gamma sarcoglycan , beta dystroglycan , and laminin alpha 2 , and results were correlated with clinical phenotype . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystrophin , utrophin and dystroglycan are present not only in muscle but also in brain . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Like dystrophin , it is composed of three regions : an N terminal region that binds actin filaments , a large central region with triple coiled coil repeats , and a C terminal region that interacts with components in the dystroglycan protein complex at the plasma membrane . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The cytoplasmic domain of beta dystroglycan was co localized with Dp 116 , the Schwann cell specific isoform of dystrophin , in the abaxonal Schwann cell cytoplasm adjacent to the outer membrane . beta dystroglycan bound to Dp 116 mainly via the 15 C terminal amino acids of its cytoplasmic domain , but these amino acids were not solely responsible for the interaction of these two proteins . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The overall approach lends itself to elucidating the developing function of other structural and adhesive components of cells , particularly skeletal muscle cells with specialized components , such as the spectrin homolog dystrophin and its membrane linked receptor dystroglycan . . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
As a result , dystroglycan and integrin redistribute into a reciprocal network as do cortical cytoskeleton components vinculin and dystrophin . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
It was revealed that ( 1 ) dystrophin , spectrin , beta dystroglycan , and sarcoglycans on the cell surface were decreased , ( 2 ) regular arrangement of the sarcoplasmic reticulum was lost , and ( 3 ) some capillaries were degenerated . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystroglycan was originally characterized in skeletal muscle as an integral component of the dystrophin glycoprotein complex , which is critical for muscle cell viability . ^^^ The smooth muscle dystroglycan complex in lung , composed of dystroglycan , dystrophin / utrophin , beta , delta , epsilon sarcoglycan , and sarcospan , can be biochemically separated from epithelial dystroglycan , which is not associated with any of the known sarcoglycans or sarcospan . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The expression and localization of dystrophin , dystroglycan , and laminin alpha 2 , a mechanical link between the actin cytoskeleton and the extracellular matrix , appears unaffected by the loss of sarcoglycan . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
WW and EF hand domains of dystrophin family proteins mediate dystroglycan binding . ^^^ We have characterized interactions between dystrophin family members and dystroglycan : cytoskeletal and transmembrane components of the complex , respectively . ^^^ 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 . ^^^ Furthermore , alpha dystrobrevin , a more distantly related dystrophin family member which lacks a WW domain but contains the EF hand domain , binds 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: Q14118 and P11532 Pubmed SVM Score :0.0
Dystrophin with dystroglycan and laminin may be a mechanical link between the actin cytoskeleton and the extracellular matrix . ^^^ By positioning itself in close proximity to dystrophin and dystroglycan , sarcoglycan may function to couple mechanical and chemical signals in striated muscle . ^^^ Sarcoglycan may be an independent signaling or regulatory module whose position in the membrane is determined by dystrophin but whose function is carried out independent of the dystrophin dystroglycan laminin axis . . ^^^
Interacting proteins: Q14118 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: Q14118 and P11532 Pubmed SVM Score :0.0
Our previous data suggested that in mouse sympathetic superior cervical ganglion ( SCG ) the dystrophin dystroglycan complex may be involved in the stabilization of the nicotinic acetylcholine receptor ( nAChR ) clusters . ^^^ Here we used SCG of dystrophic mdx mice , which express only the shorter isoforms of dystrophin ( Dys ) , to investigate whether the lack of the full length dystrophin ( Dp 427 ) could affect the localization of the dystroglycan and the alpha 3 nAChR subunit ( alpha3AChR ) at the postsynaptic apparatus . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
This further gives rise to the weak connection between dystrophin and dystroglycan complex . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Muscle biopsy showed dystrophic changes with normal expression of dystrophin and various proteins of the dystrophin associated protein complex ( sarcoglycan sub units , dystroglycan , and sarcospan ) . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystrophin associates with caveolae of rat cardiac myocytes : relationship to dystroglycan . ^^^ Caveolin ( cav ) 3 enriched , detergent insoluble membranes isolated from purified ventricular sarcolemma by density gradient fractionation were found to contain dystrophin and dystroglycan . ^^^ Further purification of cav 3 containing membranes by immunoprecipitation using anti cav 3 coated magnetic beads yielded dystrophin but not always dystroglycan . ^^^ In contrast , immunoprecipitation of membranes with anti dystrophin coated beads yielded both cav 3 and dystroglycan . ^^^ These results suggest that a subpopulation of dystrophin molecules interacts with cardiac myocyte caveolae in vivo and that some of the dystrophin is engaged in linking cav 3 with the dystroglycan complex . . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Here , we demonstrate that caveolin 3 directly interacts with beta dystroglycan , an integral membrane component of the dystrophin complex . ^^^ As the WW domain of dystrophin recognizes the same site within beta dystroglycan , we also demonstrate that caveolin 3 can effectively block the interaction of dystrophin with beta dystroglycan . ^^^ In this regard , interaction of caveolin 3 with beta dystroglycan may competitively regulate the recruitment of dystrophin to the sarcolemma . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Seventeen known human components ( three dystrophin related proteins , two dystrobrevins , five sarcoglycans , five syntrophins , one dystroglycan and one sarcospan ) appear to be reduced to eight in Drosophila ( one , one , three , two , one and none , respectively ) . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystroglycan is a member of the transmembrane dystrophin glycoprotein complex in muscle that binds to the synapse organizing molecule agrin . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The dystrophin associated membrane integrated protein complex anchors dystrophin in the sarcolemma of striated muscles and is composed of two glycoprotein subcomplexes , the dystroglycan and the sarcoglycan ( SG ) complexes , and a small membrane protein termed sarcospan ( SPN ) . ^^^ 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: Q14118 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 . ^^^ Understanding the assembly of this complex at the developing synapse led us to investigate , in Torpedo electrocyte , the intracellular routing and the targeting of several of its components , including dystroglycan , syntrophin , dystrophin and dystrobrevin . ^^^ Using cell fractionation , immunopurification and immuno electron microscope techniques , we show that beta dystroglycan , an integral glycoprotein that constitutes the core of the dystrophin associated protein complex localized at the innervated membrane , is transported together with acetylcholine receptor and rapsyn in post Golgi vesicles en route to the postsynaptic membrane . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
In the Schwann cell cytoplasm , beta dystroglycan is anchored to a dystrophin isoform , Dp 116 . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Results showed that DMD gene product deficiency affects the expression of dystroglycan assembly exclusively at the outer plexiform layer without an apparent effect on the other DAPs . ^^^ Then we have comparatively investigated the localization of dystrophin and dystrophin associated proteins ( DAPs ) in both strains to analyze the repercussion of the mdx ( 3Cv ) mutation on the retinal distributions of alpha / beta dystroglycan , alpha 1 syntrophin , alpha dystrobrevin , and delta / gamma sarcoglycan . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystroglycan , originally identified in muscle as a component of the dystrophin associated glycoprotein complex , is a ubiquitously expressed cell surface receptor that forms a transmembrane link between the extracellular matrix and the cytoskeleton . ^^^ 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 . ^^^ Using light microscopy , alpha dystroglycan was found in neurons of the cerebral cortex , hippocampus , olfactory bulb , basal ganglia , thalamus , hypothalamus , brainstem and cerebellum , where dystrophin and its truncated isoforms are also known to be present . ^^^ 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: Q14118 and P11532 Pubmed SVM Score :0.0
Dystrophin links the actin cytoskeleton to the dystroglycan complex in the plasma membrane as part of the linkage between the cytoskeleton and the extracellular matrix . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Epitopes in the interacting regions of beta dystroglycan ( PPxY motif ) and dystrophin ( WW domain ) . ^^^ The Duchenne muscular dystrophy protein , dystrophin , associates with the muscle membrane via beta dystroglycan , the WW domain of dystrophin interacting with a PPxY motif in beta dystroglycan . ^^^ A recent three dimensional structure confirms that the two epitopes are adjacent in the dystrophin dystroglycan complex , highlighting the question of how the two interacting motifs can also be accessible to antibodies during immunolocalization in situ . . ^^^
Interacting proteins: Q14118 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 ) . ^^^ In brain , dystroglycan and dystrophin are expressed on neurons and astrocytes , and some muscular dystrophies cause cognitive dysfunction ; however , no extracellular binding partner for neuronal dystroglycan is known . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The extracellular alpha dystroglycan is a membrane associated , highly glycosylated protein that binds to various extracellular matrix molecules , whereas the transmembrane beta dystroglycan binds , via its cytosolic domain , to dystrophin and many other proteins . alpha and beta Dystroglycan interact tightly but noncovalently . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystroglycan contributes to the formation of multiple dystrophin like complexes in brain . ^^^ In muscle , dystrophin anchors a complex of proteins at the cell surface which includes alpha dystroglycan , beta dystroglycan , syntrophins and dystrobrevins . ^^^ In the present study , we show that alpha dystroglycan and dystrophin are localized to large neurones in cerebral cortex , hippocampus , cerebellum and spinal cord . ^^^ Furthermore , we show that dystroglycan is a member of three distinct dystrophin containing complexes . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystrophin isoforms together with dystroglycan , a transmembrane protein which in turn binds to extracellular matrix , are the core of a complex of proteins , the dystrophin associated protein ( DAP ) complex , which also comprises cytoplasmic elements like dystrobrevin . ^^^ Transcripts for the full length dystrophin , Dp 427 , and the short isoform , Dp 71 , as well as for alpha dystrobrevin 2 increased in parallel with days in culture after RA stimulation , while dystroglycan , alpha dystrobrevin 1 and 3 , and beta dystrobrevin were constitutively expressed . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystroglycan is not required for localization of dystrophin , syntrophin , and neuronal nitric oxide synthase at the sarcolemma but regulates integrin alpha 7B expression and caveolin 3 distribution . ^^^ Dystroglycan is part of the dystrophin associated protein complex , which joins laminin in the extracellular matrix to dystrophin within the subsarcolemmal cytoskeleton . ^^^ We have investigated how mutations in the components of the laminin dystroglycan dystrophin axis affect the organization and expression of dystrophin associated proteins by comparing mice mutant for merosin ( alpha ( 2 ) laminin , dy ) , dystrophin ( mdx ) , and dystroglycan ( Dag 1 ) using immunohistochemistry and immunoblots . ^^^ We report that syntrophin and neuronal nitric oxide synthase are depleted in muscle fibers lacking both dystrophin and dystroglycan . ^^^ Some fibers deficient in dystroglycan , however , localize dystrophin at the cell surface at levels similar to that in wild type muscle . ^^^
Interacting proteins: Q14118 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: Q14118 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: Q14118 and P11532 Pubmed SVM Score :0.0
We used cultured hippocampal neurons to analyze the distribution , regulation , and role in synaptogenesis of the major transmembrane component of the DGC , dystroglycan ; one of its extracellular ligands , agrin ; and one of its cytoskeletal binding partners , dystrophin . alpha Dystroglycan , beta dystroglycan , and dystrophin clustered at a subset of inhibitory synapses containing GABA ( A ) R subunits alpha 1 , alpha 2 , and gamma 2 , and the inhibitory receptor anchoring protein gephyrin . ^^^ Analysis of gephyrin / , agrin / , and mdx mouse hippocampal neurons in culture indicated that synaptic clustering of dystroglycan occurs independently of gephyrin , agrin , and dystrophin . ^^^ In dystroglycan deficient neurons , cultured from a conditional mutant strain , GABAergic synapses differentiated with clusters of gephyrin and GABA ( A ) R apposed to synaptic terminals , but these synapses did not contain detectable dystrophin . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystroglycan , a central component of the DGC , serves as a laminin receptor via its extracellular alpha subunit , and interacts with dystrophin ( and thus the actin cytoskeleton ) through its integral membrane beta subunit . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
It was originally characterized as a member of dystrophin associated protein complex in muscle but , unlike other proteins of this complex , mutations in the dystroglycan gene have not been implicated as a cause of muscular dystrophies . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Expression of the sarcoglycans , dystroglycan and dystrophin has been confirmed by western blotting . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystroglycan associated intracellular proteins such as dystrophin , dystrobrevin , sarcoglycans , plectin and caveolin 3 are responsible for causing severe ( Duchenne type ) and moderate forms ( Becker , LGMDs ) . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Together , the sarcoglycans form a heteromeric complex that interacts with dystrophin , dystroglycan , and filamin C to form a mechanosignaling complex . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystroglycan and muscular dystrophies related to the dystrophin glycoprotein complex . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystroglycan is a component of the dystrophin glycoprotein complex that is cleaved into two polypeptides by an unidentified protease . ^^^
Interacting proteins: Q14118 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: Q14118 and P11532 Pubmed SVM Score :0.0
Previous studies have implicated dystroglycan in basement membrane formation and as a crucial link between dystrophin and laminin in muscle . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Both proteins serve as anchors for the so called dystrophin associated protein complex ( DPC ) , composed of isoforms of syntrophin , dystroglycan and dystrobrevin . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The dystrophin associated protein complex ( DPC ) consisting of syntrophin , dystrobrevin , and dystroglycan isoforms is associated either with dystrophin or its homolog utrophin . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
In this study , we investigated the proteolysis of beta dystroglycan in the biopsied skeletal muscles of various human muscular diseases , including sarcoglycanopathy , Duchenne muscular dystrophy ( DMD ) , Becker muscular dystrophy , Fukuyama congenital muscular dystrophy , Miyoshi myopathy , LGMD2A , facioscapulohumeral muscular dystrophy , myotonic dystrophy and dermatomyositis / polymyositis . ^^^ We show that the 30 kDa fragment of beta dystroglycan is increased significantly in sarcoglycanopathy and DMD , but not in the other diseases . ^^^ We propose that the proteolysis of beta dystroglycan may contribute to skeletal muscle degeneration by disrupting the link between the ECM and cell membrane in sarcoglycanopathy and DMD . . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The myofiber detachment phenotype of Dystroglycan deficient embryos was not suppressed , suggesting that Dystrophin and Dystroglycan play distinct roles in muscle formation and maintenance of muscle integrity . . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Cryosections of these biopsies were stained with antibodies against the costameric proteins of the focal adhesion complex ( vinculin , talin and integrin beta ( 1 ) ) , the dystroglycan complex ( dystrophin and beta dystroglycan ) and the spectrin based membrane cytoskeleton ( beta spectrin ) . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
C . elegans dystroglycan DGN 1 functions in epithelia and neurons , but not muscle , and independently of dystrophin . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Regarding Duchenne muscular dystrophy ( DMD ) overexpression of the synaptic CT GalNAc transferase in the sarcolemma of mdx animals was shown to induce the appearance of the CT antigen on the dystroglycan expressed at the sarcolemma . ^^^
Interacting proteins: Q14118 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 . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystrophin is an essential component in the assembly and maintenance of the dystrophin associated protein complex ( DAPC ) , which includes members of the dystroglycan , syntrophin , sarcoglycan and dystrobrevin protein families . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The absence of dystrophin leads to a dramatic reduction of the dystrophin associated proteins ( 156DAG , 59DAP , 50DAG , 43DAG and 35DAG ) in the sarcolemma of patients with Duchenne muscular dystrophy and mdx mice . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Dystrophin associated proteins are comprised of an extracellular glycoprotein of 156 kDa ( 156DAG ) , transmembrane glycoproteins of 50 kDa ( 50DAG ) , 43 kDa ( 43DAG ) and 35 kDa ( 35DAG ) , and a cytoskeletal protein of 59 kDa ( 59DAP ) . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Therefore , we conclude that the dystrophin associated protein , A3a , is retained in DMD muscles . . ^^^ A dystrophin associated glycoprotein , A3a ( one of 43DAG doublets ) , is retained in Duchenne muscular dystrophy muscle . ^^^ We determined several internal amino acid sequences of dystrophin associated glycoprotein , A3a ( one of the 43DAG doublets ) , of rabbit skeletal muscle . ^^^
Interacting proteins: Q14118 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 . . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Distribution of dystrophin isoforms and dystrophin associated proteins 43DAG ( A3a ) and 50DAG ( A 2 ) in various monkey tissues . ^^^ To determine the distributions of two known dystrophin isoforms derived from the 3 ' part of the dystrophin gene and of the dystrophin associated proteins [ 50DAG ( A 2 ) and 43DAG ( A3a ) ] by immunoblot analysis , we examined various monkey tissues [ skeletal ( quadriceps ) , cardiac ( left ventricle ) , and smooth ( aorta and uterus ) muscles , lung , liver , central nervous system ( cerebrum , cerebellum , and spinal cord ) , and peripheral nerve ( sciatic nerve ) ] . ^^^
Interacting proteins: Q14118 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 . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Major progress has also taken place in the characterization of the dystrophin associated glycoprotein ( DAG ) complex and in the understanding of its role in anchoring dystrophin to the plasmalemma and providing a link to the extracellular matrix . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The transmembrane 43K and extracellular 156K dystrophin associated glycoproteins ( DAGs ) are encoded by a single messenger RNA and the extracellular 156K DAG binds laminin . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
A severe muscular dystrophy patient with an internally deleted very short ( 110 kD ) dystrophin : presence of the binding site for dystrophin associated glycoprotein ( DAG ) may not be enough for physiological function of dystrophin . ^^^ These results suggest that an extremely short dystrophin lacking the entire actin binding site in the N terminus can not function properly even if the protein possesses the putative DAG binding cysteine rich and the C terminal domains , and still has an ability to associate with sarcolemmal membrane . . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The distribution and expression of dystrophin and three of the dystrophin associated glycoproteins ( DAG ) , alpha dystroglycan ( 156 kDa DAG ) , beta dystroglycan ( 43 kDa DAG ) and adhalin ( 50 kDa DAG ) in rat skeletal muscle were studied during a controlled cycle of degeneration and regeneration induced by the injection of a snake venom . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
A certain membrane instability is probably responsible for muscle fiber necrosis , because defects in membrane proteins have been proposed to associate with progressive muscular dystrophies including dystrophin in DMD , a 50 KD subunit of dystrophin associated glycoprotein ( DAG ) in severe childhood autosomal recessive muscular dystrophy ( SCARMD ) , and subunit M of laminin ( merosin ) in congenital muscular dystrophy and dy mouse . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
This is strong evidence supporting Ervasti & Campbell ' s model in which the DAG complex is bound close to the C terminus of dystrophin and in which the DAG complexes are separated by approximately the length of the dystrophin rod . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
It was recently shown that one component of this complex , the 50 kDa dystrophin associated glycoprotein ( 50 DAG or adhalin ) , is deficient in severe childhood autosomal recessive muscular dystrophy with DMD like phenotype ( SCARMD ) . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The relationship of a member of the transmembrane dystrophin associated glycoprotein ( DAG ) complex to acetylcholine receptors ( AChRs ) was investigated using immunofluorescence techniques at rat neuromuscular junctions ( NMJs ) viewed en face . ^^^ The region of highest 43 K DAG ( 43DAG ) labelling projected beyond the AChRs by approximately 0 . 3 microns , as does that for dystrophin . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
The potential role of the sarcoglycan subcomplex , especially of alpha sarcoglycan ( adhalin ) , as part of the DC in holding of NOS 1 in the sarcolemmal position was examined by carrying out a comparative study on the distribution of NOS 1 , dystrophin , dystrophin associated glycoproteins ( DAG ) and alpha sarcoglycan in various skeletal muscles of non mammals . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Immunohistochemistry revealed normal dystrophin , dystrophin associated glycoproteins ( DAG ) and merosin . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Muscular immunocytochemical studies showed a normal staining for dystrophin and all dystrophin related glycoproteins ( including 43 and 50 DAG ) . ^^^
Interacting proteins: Q14118 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: Q14118 and P11532 Pubmed SVM Score :0.0
A membrane hypothesis to explain fiber necrosis seems to be confirmed by the discovery of dystrophin and dystrophin associated glycoprotein ( DAG ) ; both are located along the muscle surface membrane . ^^^
Interacting proteins: Q14118 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: Q14118 and P11532 Pubmed SVM Score :0.0
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 . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Its N terminal domain binds to F actin and its C terminus binds to the dystrophin associated glycoprotein ( DAG ) complex in the membrane . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
Defects of the glycoprotein complex associated to dystrophin ( DAG ) are rare skeletal muscle diseases with possible cardiac involvement . ^^^ By candidate gene screening , the molecular diagnosis can be provided for dystrophin , DAG , mitochondrial DNA , actin and desmin gene defects . . ^^^
Interacting proteins: Q14118 and P11532 Pubmed SVM Score :0.0
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