SELECTIVE DEFECT OF SARCOGLYCAN COMPLEX IN SEVERE CHILDHOOD AUTOSOMAL RECESSIVE MUSCULAR-DYSTROPHY MUSCLE

被引：70

作者：

MIZUNO, Y ^{[1
]}

NOGUCHI, S ^{[1
]}

YAMAMOTO, H ^{[1
]}

YOSHIDA, M ^{[1
]}

SUZUKI, A ^{[1
]}

HAGIWARA, Y ^{[1
]}

HAYASHI, YK ^{[1
]}

ARAHATA, K ^{[1
]}

NONAKA, I ^{[1
]}

HIRAI, S ^{[1
]}

OZAWA, E ^{[1
]}

机构：

[1] GUNMA UNIV,DEPT NEUROL,MAEBASHI,GUMMA 371,JAPAN

来源：

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS | 1994年 / 203卷 / 02期

关键词：

D O I：

10.1006/bbrc.1994.2278

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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 tile disease with a selective defect of the sarcoglycan complex. (C) 1994 Academic Press, Inc.

引用

页码：979 / 983

页数：5

共 9 条

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[3] DISTRIBUTION OF DYSTROPHIN ISOFORMS AND DYSTROPHIN-ASSOCIATED PROTEINS 43DAG (A3A) AND 50DAG (A2) IN VARIOUS MONKEY TISSUES
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