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  • Title: Selective defect of sarcoglycan complex in severe childhood autosomal recessive muscular dystrophy muscle.
    Author: Mizuno Y, Noguchi S, Yamamoto H, Yoshida M, Suzuki A, Hagiwara Y, Hayashi YK, Arahata K, Nonaka I, Hirai S.
    Journal: Biochem Biophys Res Commun; 1994 Sep 15; 203(2):979-83. PubMed ID: 8093083.
    Abstract:
    Dystrophin-associated glycoprotein complex is classified into two subcomplexes: the dystroglycan complex (156DAG and 43DAG) and the sarcoglycan complex (50DAG, A3b, and 35DAG). Severe childhood autosomal recessive muscular dystrophy (SCARMD) was first reported to result from a deficiency of 50DAG. 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. Therefore, SCARMD is the disease with a selective defect of the sarcoglycan complex.
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