| Interacting proteins: Q92629 and Q16586 |
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 . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| We recently reported that the dystrophin associated glycoprotein ( DAG ) complex is biochemically divided into two subcomplexes : one is the dystroglycan complex comprised of 156DAG and 43DAG and the other is the sarcoglycan complex comprised of 50DAG , A3b , and 35DAG . ^^^ In the present study , we examined the striated muscles of the dystrophic hamster with anti A3b antibody in addition to anti 50DAG , anti 43DAG , anti 35DAG , anti dystrophin , and anti laminin antibodies by both immunohistochemistry and immunoblot analysis and found that 50DAG , A3b , and 35DAG are selectively lost . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| In particular , we found that the glycoprotein complex stated above was dissociated into two distinct groups : one composed of 156DAG and 43DAG ( A3a ) and the other composed of 50DAG , 35DAG and A3b . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
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 . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
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 . ^^^ On the other hand , in the dystrophic hamster 50DAG ( A 2 ) and 35DAG ( A 4 ) were selectively defective , and 43DAG ( A3a ) was also decreased , although to a lesser degree . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| Expression of dystrophin associated protein 35DAG ( A 4 ) and 50DAG ( A 2 ) is confined to striated muscles . ^^^ On the basis of these results , it is concluded that 35DAG and 50DAG are striated muscle specific and may be important in the pathogenesis of Duchenne muscular dystrophy . . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| A group of transmembrane proteins known as alpha ( adhalin ) beta , gamma and delta sarcoglycan are deficient in autosomal recessive limb girdle muscular dystrophy , and the extracellular matrix protein merosin ( alpha 2 laminin ) , is deficient in a subset of patients with congenital muscular dystrophy . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| Pathogenetic mutations involving one gene for sarcoglycan complex components were identified in 13 patients : alpha sarcoglycan in seven , beta sarcoglycan in two , gamma sarcoglycan in four , and none in the delta sarcoglycan gene . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| We investigated the expression of alpha sarcoglycan , beta sarcoglycan , gamma sarcoglycan , and delta sarcoglycan immunohistochemically in three patients with mutations of the alpha sarcoglycan gene and a patient with a mutation of the gamma sarcoglycan gene . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| We demonstrate that the interaction between beta and delta sarcoglycan is resistant to high concentrations of SDS and alpha sarcoglycan is less tightly associated with other members of the complex . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
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 . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| To investigate mechanisms in the pathogenesis of cardiomyopathy associated with mutations of the dystrophin glycoprotein complex , we analyzed genetically engineered mice deficient for either alpha sarcoglycan ( Sgca ) or delta sarcoglycan ( Sgcd ) . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| Without gamma sarcoglycan , beta and delta sarcoglycan are unstable at the muscle membrane and alpha sarcoglycan is severely reduced . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
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 ) . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
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 . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| 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 . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
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 . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| These results suggest that epsilon sarcoglycan serves a function similar to that of alpha sarcoglycan and that residual beta , gamma , and delta sarcoglycan seen in mutant mice and alpha sarcoglycan deficient patients is due to its association with epsilon sarcoglycan . . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| The classification of families was as follows : calpainopathy 7 , dysferlinopathy 3 , alpha sarcoglycan deficiency 2 , beta sarcoglycan deficiency 7 , gamma sarcoglycan deficiency 5 , delta sarcoglycan deficiency 1 , and merosinopathy 2 . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
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: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| They are : calpain 3 ( LGMD2A ) , dysferlin ( LGMD2B ) , alpha sarcoglycan ( LGMD2D ) , beta sarcoglycan ( LGMD2E ) , gamma sarcoglycan ( LGMD2C ) , delta sarcoglycan ( LGMD2F ) , telethonin ( LGMD2G ) , TRIM 32 ( LGMD2H ) , fukutin related protein ( LGMD2I ) and titin ( LGMD2J ) . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| Our results , acquired with an immunofluorescence study on adult human skeletal muscle affected by LGMD type 2D and 2C , showed that in LGMD2D : a ) alpha sarcoglycan staining is severely reduced ; b ) the beta gamma delta sarcoglycan subunit and all tested integrins staining are clearly detectable ; c ) filamin 2 is normal and shows a costameric distribution . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| It is hypothesized that epsilon sarcoglycan might replace alpha sarcoglycan in smooth muscle , forming a novel sarcoglycan subcomplex consisting of epsilon , beta , gamma , and delta sarcoglycan . ^^^ On this basis , growing evidence suggests that there are two types of sarcoglycan complex ; one , in skeletal and cardiac muscle , consisting of alpha , beta , gamma and delta sarcoglycan ; and the other , in smooth muscle , containing beta , delta , zeta and epsilon sarcoglycan . epsilon sarcoglycan may be substituted for alpha sarcoglycan in a subset of striated muscle complexes . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| We have designed Multiplex Amplifiable Probe Hybridization ( MAPH ) probes for 28 exons of the sarcoglycan genes SGCA , SGCB , SGCG , and SGCD . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| To get a closer view on the differentiating molecular events responsible for the muscular dystrophies , we have carried out a comparative gene expression profiling of hindlimb muscles of the following mouse models : dystrophin deficient ( mdx , mdx ( 3cv ) ) , sarcoglycan deficient ( Sgca null , Sgcb null , Sgcg null , Sgcd null ) , dysferlin deficient ( Dysf null , SJL ( Dysf ) ) , sarcospan deficient ( Sspn null ) , and wild type ( C57Bl / 6 , C57Bl / 10 ) mice . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| Mutations in SGCA , SGCB , SGCG and SGCD genes are associated with LGMD 2D , 2E , 2C and 2F , respectively . ^^^ |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: Q92629 and Q16586 |
Pubmed |
SVM Score :0.0 |
| NA |
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