Selective defect of sarcoglycan complex in severe childhood autosomal recessive muscular dystrophy muscle
Y Mizuno, S Noguchi, H Yamamoto, M Yoshida… - Biochemical and …, 1994 - Elsevier
Y Mizuno, S Noguchi, H Yamamoto, M Yoshida, A Suzuki, Y Hagiwara, YK Hayashi…
Biochemical and biophysical research communications, 1994•ElsevierDystrophin-associated glycoprotein complex is classified into two subcomplexes: the
dystroglycan complex (156DAC 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 …
dystroglycan complex (156DAC 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 …
Abstract
Dystrophin-associated glycoprotein complex is classified into two subcomplexes: the dystroglycan complex (156DAC 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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