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Sarcoglycanopathy: The establishment of its concept and the recent progress in the studies on its molecular mechanism Mikiharu YOSHIDA 1 1Department of Cell Biology, National Institute of Neuroscience, NCNP Keyword: サルコグリカン , sarcoglycan , ジストロフィン結合タンパク質 , dystrophin-associated protein , 病態の分子機構 , molecular mechanism of pathophysiology , 筋ジストロフィー , muscular dystrophy pp.178-188
Published Date 2000/4/10
DOI https://doi.org/10.11477/mf.1431901137
  • Abstract
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Sarcoglycanopathy is a Duchenne-like muscular dystrophy inherited in an autosomal recessive mode and a concept of disease recently developed. Sarcoglycanopathy is so called, because the absence or marked reduction of the sarcoglycan complex from sarcolemma results in muscular dystrophic phenotypes. This complex named sarcoglycan is composed of four dystrophin-associated proteins termed a-to a -sarcoglycans and spanning the sarcolemma. Sarcoglycanopathy is originated in the disease called severe childhood autosomal recessive muscular dystrophy or SCARMD.


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電子版ISSN 1882-1243 印刷版ISSN 0001-8724 医学書院

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