Alpha Sarcoglycan Proteins

Protein Information

The sarcoglycan transmembrane proteins are members of the dystrophin complex. Sarcoglycans cluster together to form a complex, which is localized in the cell membrane of skeletal, cardiac, and smooth muscle fibers. Four sarcoglycan subunit proteins, designated alpha-, beta-, gamma- and delta-sarcoglycan, form a complex on the skeletal muscle cell surface membrane. A genetic defect in any one of these proteins causes the loss or marked decrease of the whole sarcoglycan complex, which is observed in the autosomal recessive muscular dystrophy, sarcoglycanopathy. In smooth muscle, beta- and delta-sarcoglycans are associated with epsilon-sarcoglycan, a glycoprotein homologous to alpha-sarcoglycan. Additionally, a complete deficiency in delta-sarcoglycan is the cause of the Syrian hamster BIO.14 cardiomyopathy.

Synonyms

50 kDa dystrophin-associated glycoprotein; 50DAG; 50kD DAG; adhalin; ADL; Alpha-sarcoglycan; Alpha-SG; Asg; DAG2; DMDA2; Dystroglycan 2; Dystroglycan2; Dystroglycan-2; LGMD2D; limb girdle muscular dystrophy 2D; sarcoglycan alpha; sarcoglycan, alpha (50kDa dystrophin-associated glycoprotein); sarcoglycan, alpha (dystrophin-associated glycoprotein); SCARMD1; SG alpha; SG-alpha; Sgca