| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| There was complete absence of the 50kDa dystrophin associated glycoprotein ( alpha sarcoglycan ) . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunohistochemical analyses of dystrophin . merosin , and adhalin were normal . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin , laminin alpha 2 , and adhalin stains revealed normal immunoreactivity . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| In muscle , beta dystroglycan was found to be more tightly associated with dystrophin than alpha sarcoglycan . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Reducing bodies were strongly immunoreactive with antibodies to dystrophin , alpha sarcoglycan , vimentin and ubiquitin . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Apparent in vivo DMD was accurately estimated ( P greater than . 10 ) by INVITRO , ADL and APLPST with Diet 1 . ^^^ |
|
| Interacting proteins: Q16586 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: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Recently , we have demonstrated the specific deficiency of the 50 kDa dystrophin associated glycoprotein ( 50DAG ) in severe childhood autosomal recessive muscular dystrophy with Duchenne like phenotype ( SCARMD or AR DLMD ) , a disease first reported in Tunisia and now presumed to be prevalent in North Africa and the Middle East . ^^^ Without the analysis of both dystrophin and 50DAG , isolated male patients with this condition could be undiagnosed or misdiagnosed as having Duchenne or severe Becker muscular dystrophy . ^^^ We also report , for the first time , the normal expression of the 50DAG and other dystrophin associated proteins in one negroid and 2 caucasoid Brazilian patients with a phenotype indistinguishable from that of AR DLMD with 50DAG deficiency . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| It has been previously shown in Tunisian and Algerian families that the locus for SCARMD maps to the proximal part of 13q , and in Algerian families that the disease is associated with deficiency of the 50 kDa dystrophin associated glycoprotein ( 50DAG ) . ^^^ |
|
| Interacting proteins: Q16586 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 ) . ^^^ We examined muscles from five SCARMD patients and found that dystrophin and 43DAG were present in almost normal levels while 35DAG and the newly identified protein A3b in addition to 50DAG were absent or greatly reduced . ^^^ |
|
| Interacting proteins: Q16586 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 . . ^^^ 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: Q16586 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 ) ] . ^^^ Dystrophin associated protein , 43DAG , was detected in all the tissues examined , but 50DAG was detected only in skeletal and cardiac muscles . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Selective defect in dystrophin associated glycoproteins 50DAG ( A 2 ) and 35DAG ( A 4 ) in the dystrophic hamster : an animal model for severe childhood autosomal recessive muscular dystrophy ( SCARMD ) . ^^^ Antibodies against dystrophin , utrophin and DAGs including 50DAG ( A 2 ) , 43DAG ( A3a ) and 35DAG ( A 4 ) were employed for the examination . ^^^ |
|
| Interacting proteins: Q16586 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 ) . ^^^ In sharp contrast to skeletal muscle , however , full size dystrophin and 50DAG were undetectable in peripheral nerve . ^^^ |
|
| Interacting proteins: Q16586 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 . ^^^ The 50 kd dystrophin associated glycoprotein ( 50DAG or adhalin ) in the skeletal muscle has been shown to be deficient in patients with severe childhood autosomal recessive muscular dystrophy prevalent in North Africa . ^^^ 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: Q16586 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: Q16586 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: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Adhalin deficiency likely causes disruption of the muscle cell membrane , resulting in dystrophic changes in the skeletal muscle similar to dystrophin deficiency in Duchenne muscular dystrophy . . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Primary adhalin deficiency as a cause of muscular dystrophy in patients with normal dystrophin . ^^^ Recently , a French family with 4 young siblings showing a muscular dystrophy of unknown progression was shown to have a primary deficiency of `` adhalin , ' ' the 50 kd dystrophin associated protein . ^^^ Our results suggest that primary adhalin deficiency in patients with muscular dystrophy but normal dystrophin is relatively infrequent , and that adhalin deficient patients are not restricted to the French population . . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| In severe childhood autosomal recessive muscular dystrophy , a selective deficiency of adhalin ( 50 kd glycoprotein ) also causes dysfunction of the dystrophin glycoprotein complex . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| BACKGROUND : In skeletal muscle , dystrophin exists in a large oligomeric complex tightly associated with several novel sarcolemmal proteins , including the 50 kDa transmembrane glycoprotein called adhalin . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| The expression of both adhalin splice forms is exclusively restricted to striated muscle , unlike other components of the dystrophin glycoprotein complex . . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Genetic heterogeneity of severe childhood autosomal recessive muscular dystrophy with adhalin ( 50 kDa dystrophin associated glycoprotein ) deficiency . ^^^ A striking feature of this disease is the isolated deficiency of adhalin , a sarcolemmal 50 kDa dystrophin associated glycoprotein . ^^^ In the 4 cases adhalin was completely absent in muscle sections , whereas dystrophin and the other members of the dystrophin associated protein complex were normal , except for the 35 kDa dystrophin associated glycoprotein which was decreased as usually observed in SCARMD . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin is associated with several novel sarcolemmal proteins , including a laminin binding extracellular glycoprotein of 156 kD ( alpha dystroglycan ) and a transmembrane glycoprotein of 50 kD ( adhalin ) . ^^^ |
|
| Interacting proteins: Q16586 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 ) . ^^^ Deficiency of the 50 kDa dystrophin associated glycoprotein ( adhalin ) in severe autosomal recessive muscular dystrophies in children native from European countries . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Expression of the dystrophin associated glycoproteins adhalin , alpha dystroglycan , and beta dystroglycan were normal in the three patients . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Another boy of the same age ( from a family characterized by early onset and slower progression ) had normal dystrophin and adhalin . ^^^ |
|
| Interacting proteins: Q16586 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: Q16586 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 . ^^^ Following surgical denervation , the levels of adhalin and beta dystroglycan dramatically increased at the extrajunctional sarcolemma with AChR , suggesting that their association is independent of dystrophin . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| The dystrophin associated proteins are classified into three groups : ( 1 ) alpha and beta dystroglycan , ( 2 ) adhalin , 35DAG and A3b , and ( 3 ) members of the syntrophin family . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| In the present study we addressed the question of whether two major dystrophin associated integral membrane proteins of the muscular plasma membrane , beta dystroglycan and adhalin , are also present in photoreceptor synaptic complexes . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Deficiencies of adhalin in a particular form of limb girdle muscular dystrophy , and of merosin in a particular form of congenital muscular dystrophy as well as the newly discovered principle of abnormal tri nucleotide repeats in myotonic dystrophy are evidence of progress that has also amplified the notion of the dystrophinopathies that the protein deficient muscular dystrophies can now be considered examples of contributions of the dystrophin glycoprotein complex across the muscle fiber plasma membrane . . ^^^ |
|
| Interacting proteins: Q16586 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: Q16586 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: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Beta dystroglycan of 43 kDa , together with the 50 kDa protein adhalin and three other dystrophin associated glycoproteins of 25 , 35 and 43 kDa form this sarcolemmal complex which binds to the cysteine rich domain of dystrophin . ^^^ |
|
| Interacting proteins: Q16586 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: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| In the absence of dystrophin , in Duchenne muscular dystrophy ( DMD ) and the mdx mouse , levels of adhalin , alpha dystroglycan and other components of the complex , are severely reduced , and it has been speculated that this might be an important factor in precipitating myofibre necrosis . ^^^ In DMD the labelling of adhalin and alpha dystroglycan is severely reduced quantitatively ( although the vestige that remains is positioned normally ) but merosin is expressed normally , showing that its incorporation is independent of that of dystrophin and its associated proteins . . ^^^ Adhalin and alpha dystroglycan are two components of a complex of proteins that , in conjunction with dystrophin , provide a link between the subsarcolemmal cytoskeleton and the basal lamina of the extracellular matrix of skeletal muscle . ^^^ From biochemical data it might be predicted that adhalin and alpha dystroglycan are positioned more peripherally in the muscle cell than dystrophin and more proximal than merosin . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Electron microscopic observations of triple immunogold labelling for dystrophin , beta dystroglycan and adhalin in human skeletal myofibers . ^^^ Adhalin ( 50 kDa dystrophin associated glycoprotein ) and beta dystroglycan ( 43 kDa dystrophin associated glycoprotein ) are the transmembrane components of the normal muscle plasma membrane , and beta dystroglycan has been demonstrated to bind dystrophin at the inside surface of normal muscle plasma membrane . ^^^ This investigation was undertaken to test whether the epitopes of dystrophin , beta dystroglycan and adhalin are closely associated with each other by using triple immunogold labelling electron microscopy on normal human skeletal myofibers . ^^^ However , closely associated signals of two epitopes of dystrophin and beta dystroglycan , dystrophin and adhalin , or adhalin and beta dystroglycan were frequently observed . ^^^ These ultrastructural findings are consistent with biochemical evidence implying that dystrophin , beta dystroglycan and adhalin are closely associated with each other at the normal muscle plasma membrane . . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| We report adhalin deficiency in 8 patients with clinically diagnosed muscular dystrophy , dystrophic histopathological features , high plasma creatine kinase levels , normal expression of dystrophin , and marked variability of symptoms . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| The ultrastructural localization of adhalin and its relations to dystrophin , beta dystroglycan , and beta spectrin were studied in normal murine skeletal myofibers . ^^^ The close association of adhalin with dystrophin or beta dystroglycan was demonstrated by formation of doublets by signals of antibodies of adhalin with those of dystrophin or beta dystroglycan and was confirmed by statistical analyses . ^^^ This study demonstrated that the location of adhalin is close to that of dystrophin and beta dystroglycan at the muscle plasma membrane . . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Two siblings with a congenital muscular dystrophy and severe mental retardation which was not due to dystrophin , merosin , or adhalin deficiency are described . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| The proper localization of adhalin to the muscle cell membrane was observed only in late stages of myotube maturation , coincident with the re distribution of caveolin 3 and dystrophin . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| We investigated in situ DNA fragmentation by the TUNEL method and expression of apoptosis related proteins immunohistochemically in 14 children suffering from deficiencies of dystrophin , adhalin , and merosin , and found TUNEL positive chromatin cleavage of muscle fibre nuclei in about 10 % of non necrotic muscle fibres . ^^^ |
|
| Interacting proteins: Q16586 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: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunocytochemical deficiency of alpha sarcoglycan ( adhalin ) in the skeletal muscle that is associated with normal dystrophin expression has been called adhalinopathy . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Additionally , members of the sarcolemmal dystrophin glycoprotein complex ( i . e . , dystrophin , adhalin , beta 1 dystroglycan ) immunolocalize in the cytosol of differentiating myoblasts , whereas anti dystrophin and anti beta 1 dystroglycan clearly delineate the sarcolemma in myotubes . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Laminin alpha 2 ( merosin ) , beta dystroglycan , alpha sarcoglycan ( adhalin ) , and dystrophin expression in congenital muscular dystrophies : an immunohistochemical study . ^^^ Muscle biopsies of 13 congenital muscular dystrophy ( CMD ) patients were investigated for the expression of laminin alpha 2 ( merosin ) , beta dystroglycan , alpha sarcoglycan ( adhalin ) and dystrophin . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| The histological pattern showed a destructed fascicular architecture in agreement with severe muscular dystrophy , normal staining with anti dystrophin monoclonal antibodies and abnormal staining pattern with anti adhalin antibodies . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Four patients underwent a muscle biopsy that was processed for routine enzyme histochemistry and immunocytochemical analyses for dystrophin and adhalin ( alpha sarcoglycan ) . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| The only known disease gene is the dystrophin gene causing 10 linked dilated cardiomyopathy , but other cytoskeletal proteins , such as adhalin , could be involved . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunohistochemistry revealed normal expression of the dystrophin glycoprotein complex ( DGC ) , including dystrophin , beta dystroglycan , alpha ( adhalin ) , beta , gamma , and delta sarcoglycan , laminin alpha 2 chain ( merosin ) and syntrophin . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| This work describes the culture of Sol 8 cell line that expresses neither dystrophin nor adhalin , a dystrophin associated protein . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| OBJECTIVE : To evaluate the potential importance of dystrophin , alpha sarcoglycan ( adhalin ) , and beta dystroglycan , by use of western blot analysis , in several breeds of dogs with dilated cardiomyopathy . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Deficiency of the 50 kDa dystrophin associated glycoprotein ( adhalin ) in an Indian autosomal recessive limb girdle muscular dystrophy patient : immunochemical analysis and clinical aspects . ^^^ Dystrophin associated proteins , beta dystroglycan showed discontinuous immunostaining in the sarcolemma and alpha sarcoglycan ( adhalin ) was totally absent , while beta , gamma , and delta sarcoglycans were highly reduced . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Muscle immunohistochemistry revealed normal dystrophin 1 and 2 staining but complete absence for adhalin , a dystrophin associated glycoprotein . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunostaining of TAs was also seen with antibodies against heat shock protein and dysferlin , but not with antibodies to lamin A , dystrophin , adhalin , beta , gamma , delta sarcoglycans , and merosin . ^^^ |
|
| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Using differentiated C2C12 skeletal myoblasts as a model system , we observe that caveolin 3 co fractionates with cytoplasmic signaling molecules ( G proteins and Src like kinases ) and members of the dystrophin complex ( dystrophin , alpha sarcoglycan , and beta dystroglycan ) , but is clearly separated from the bulk of cellular proteins . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Expression of a dystrophin sarcoglycan complex in serum deprived BC3H1 cells and involvement of alpha sarcoglycan in substrate attachment . ^^^ Thus dystrophin and DAPs , at least partly , form a complex with the focal adhesion proteins in differentiated BC3H1 cells and alpha sarcoglycan seems to modulate the function of the focal adhesion complex in these cells . . ^^^ |
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| Interacting proteins: Q16586 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 ) . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| These patients were selected on the basis of autosomal inheritance and / or the presence of normal dystrophin and / or deficiency of alpha sarcoglycan immunostaining . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| The muscle biopsies from these 50 patients showed no abnormality of dystrophin but did show diminished immunostaining for the dystrophin associated protein alpha sarcoglycan . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| METHODS : Antibody against alpha sarcoglycan was used to stain muscle biopsy specimens from 556 patients with myopathy and normal dystrophin genes ( the gene frequently deleted in 10 linked muscular dystrophy ) . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| The other cytoskeletal proteins , including dystrophin and 50 kDa alpha sarcoglycan were normally expressed around all muscle fibers . . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin , beta dystroglycan , alpha sarcoglycan , and spectrin were found normally expressed in both the junctional and extrajunctional sarcolemma of both fiber types . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| METHODS : We studied muscle biopsy specimens from 18 patients with LGMD using immunofluorescence with antibodies against dystrophin C terminus , beta dystroglycan , alpha sarcoglycan , gamma sarcoglycan , and the laminin subunits merosin , beta 1 , and gamma 1 . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunohistochemical and immunoblot screening for alpha sarcoglycan protein deficiency was performed on all muscle biopsies from patients with a progressive muscular dystrophy of unknown aetiology and normal dystrophin . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| A complex of dystrophin and its associated proteins was fully expressed in L 6 myotubes , from which anti dystrophin or anti alpha sarcoglycan co precipitated integrin alpha 5 beta 1 and other focal adhesion associated proteins vinculin , talin , paxillin , and focal adhesion kinase . ^^^ Immunostaining and confocal microscopy revealed that dystrophin , alpha sarcoglycan , integrin alpha 5 beta 1 , and vinculin exhibited overlapping distribution in the sarcolemma , especially at focal adhesion like , spotty structures . ^^^ |
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| Interacting proteins: Q16586 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 . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| In vivo , dystrophin and the dystrophin associated glycoproteins alpha sarcoglycan and beta dystroglycan are morphologically disrupted in infected myocytes . ^^^ |
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| Interacting proteins: Q16586 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 . ^^^ Together , these results demonstrate a tissue dependent variation in the sarcoglycan complex and show that epsilon sarcoglycan replaces alpha sarcoglycan as an integral component of the smooth muscle dystrophin glycoprotein complex . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunocytochemistry demonstrated normal spectrin and dystrophin , reduced alpha sarcoglycan and absent gamma sarcoglycan indicating a gamma sarcoglycanopathy . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Within the better preserved parts of the muscles , i . e . , those without signs of necrotic processes , dystrophin , spectrin , and some of the dystrophin associated proteins ( beta dystroglycan , alpha sarcoglycan , and gamma sarcoglycan ) disappeared from sarcolemma of many fibers . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| We used expression profiling to define the pathophysiological cascades involved in the progression of two muscular dystrophies with known primary biochemical defects , dystrophin deficiency ( Duchenne muscular dystrophy ) and alpha sarcoglycan deficiency ( a dystrophin associated protein ) . ^^^ We hypothesize that the abnormal Ca ( 2 ) + influx in dystrophin and alpha sarcoglycan deficient myofibers leads to altered developmental programming of developing and regenerating myofibers . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunohistochemistry demonstrated that NOS 1 was co localized with integral ( beta dystroglycan , alpha sarcoglycan ) and peripheral ( caveolin 3 , dystrophin ) members of the enlarged dystrophin complex in the irregular costameres but not with non sarcolemmal organized proteins ( myosin heavy chain , alpha actinin , desmin and sarcoplasmic reticulum located Ca2+ dependent ATPase 1 ) . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| A dystrophin associated protein , alpha sarcoglycan , has recently been shown to be an ecto ATPase ( an extracellular ATPase ) capable of regulating ATP concentrations in the space between the cardiomyocytes . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| The expression of dystrophin , alpha sarcoglycan , and beta dystroglycan during skeletal muscle regeneration : immunohistochemical and western blot studies . ^^^ We evaluated re expression of dystrophin , alpha sarcoglycan and beta dystroglycan in regenerating skeletal muscles of rats after cardiotoxin induced myonecrosis in order to understand the dynamic behaviour of these proteins during the regeneration process . ^^^ After the 14th day , dystrophin was stained conspicuously . alpha Sarcoglycan was labeled weakly at the sarcolemma of small regenerating muscle fibers on the 5th day and was labeled conspicuously after the 7th day . beta Dystroglycan was labeled moderately at the sarcolemma of regenerating muscle fibers on the 5th day and was labeled conspicuously after the 7th day . ^^^ In western blot analysis , beta dystroglycan persisted throughout the entire cycle of myonecrosis and regeneration , and re expression of alpha sarcoglycan progressed faster than that of dystrophin . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| CASE REPORT : The child presented with neonatal hypotonia , delayed motor abilities and speech , seizures , cerebral and cerebellar gyrus abnormalities with signal intensity change in the white matter by MRI , high serum level of creatinephosphokinase ( CK ) , and dystrophic skeletal muscle with normal merosin , alpha sarcoglycan and dystrophin expression . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| The amount of some of the DGC proteins , including dystrophin , beta dystroglycan , and alpha sarcoglycan , is reduced significantly in rat skeletal muscle atrophy induced by tenotomy . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin , beta , delta sarcoglycan and beta dystroglycan are first expressed in the myotome at the 4th week of human embryogenesis , followed by gamma sarcoglycan and merosin at the 6th week of development ; alpha sarcoglycan appears only at the level of the muscular fibre at the end of the embryonic period . . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| This ischemic loss of dystrophin was a specific event in that no ischemic loss of sarcolemmal alpha sarcoglycan , gamma sarcoglycan , alphaDG , or betaDG was observed . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| By immunofluorescence and Western blot analysis , we show that administration of the proteasomal inhibitor MG 132 effectively rescues the expression levels and plasma membrane localization of dystrophin , beta dystroglycan , alpha dystroglycan , and alpha sarcoglycan in skeletal muscle fibers from mdx mice . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| RESULTS : Hemodynamic parameters of CAL rats at the 8th week after CAL ( 8w CAL ) indicated heart failure . alpha Sarcoglycan ( SG ) and dystrophin in the surviving left ventricle ( surviving LV ) of 8w CAL rats decreased , whereas beta , gamma , and delta SGs remained unchanged . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Alpha sarcoglycan ( Sgca ) is a transmembrane glycoprotein of the dystrophin complex located at skeletal and cardiac muscle sarcolemma . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Oral administration of 3 mg / kg / day trandolapril or 1 mg / kg / day candesartan from the 2nd to 8th week after the left coronary artery ligation attenuated decreases in alpha sarcoglycan and dystrophin and reduced the increased proteolytic activity . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Using this approach , we have demonstrated that treatment with a well characterized proteasome inhibitor , MG 132 , is sufficient to rescue the expression of dystrophin , beta dystroglycan , and alpha sarcoglycan in skeletal muscle explants from patients with Duchenne or Becker muscular dystrophy . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| RESULTS : Dystrophin and dysferlin were normal in all ; among the patients with MD CMD , merosin deficiency was partial in nine who showed the same clinical severity as those with total deficiency ; the reduced expression of alpha sarcoglycan ( SG ) and alpha dystroglycan ( DG ) showed statistically significant correlation with severe MD CMD phenotype . ^^^ |
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| Interacting proteins: Q16586 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 . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Finally , 50 DAG mRNA is present in mdx and Duchenne muscular dystrophy ( DMD ) muscle , indicating that the down regulation of this protein in DMD and the mdx mouse is likely a post translational event . . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| The five dystrophin associated proteins ( DAPs ) , 156 DAG , 59 DAP , 50 DAG , 43 DAG , and 35 DAG , were identified in rabbit ventricular muscle and found to codistribute with dystrophin in both papillary myofibers and Purkinje fibers . ^^^ |
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| Interacting proteins: Q16586 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 ) . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| In addition , patients with impaired activities of daily living ( ADL ) , evaluated by a mobility score , had significantly decreased BMD ratios of paretic / nonparetic side than patients with improved ADL ( femoral neck 91 % versus 97 % , P < 0 . 01 and total femur 89 % versus 94 % , P < 0 . 05 ) . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| The purpose of this study was to reach a better understanding the deficits of activity of daily living ( ADL ) skills in patients with Duchenne muscular dystrophy ( DMD ) , and their influencing factors . ^^^ In conclusion , we find that DMD patients are highly dependent in carrying out ADL tasks . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| BMD , motor function , ambulation and activities of daily living ( ADL ) were assessed at 1 , 4 , 7 and 12 months after stroke onset . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| The low ADL group had a larger decrease in BMD than the high ADL group . ^^^ For the control group , the BMD rate of change on the paretic side of the femoral neck was 9 . 6 % / 3 mo for the low ADL group . ^^^ BMD loss was reduced significantly by the administration of etidronate for the low ADL group . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| In DMD subjects , manifestations of open bite were related to ADL score , sagittal shortening and transverse expansion of the dental arch and vertical overgrowth of the lower jaw . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Self reported pain and FIQ ADL among FM patients correlated with BMD femoral neck ( r ( s ) = 0 . 52 , p = 0 . 003 ) ; ( r ( s ) = 0 . 31 , p = 0 . 09 ) . ^^^ |
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| Interacting proteins: Q16586 and P11532 |
Pubmed |
SVM Score :0.0 |
| Of the 200 initial participants , 124 ( 57 men , 67 women ; 62 . 0 % ) completed all BMD measurements and answered all items about ADL in the follow up survey . ^^^ To evaluate relationships between decreased ADL and changes in BMD , annual rates of change for BMD at the lumbar spine and femoral neck were compared to changes for each ADL item ( 2 grade decrease ; 1 grade decrease ; or no change ) . ^^^ |
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