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Abnormal Expression of Dystrophin-Associated Proteins in Fukuyama-Type Congenital Muscular Dystrophy Lancet 341:521-522, Matsumura,K.,et al, 1993 See this aricle in Pubmed Article Abstract The absence of dystrophin causes Duchenne muscular dystrophy.Dystrophin is associated with a large complex of sarcoemmal glycoproteins which provises a linkage to the extracellular matrix component,laminin,and when dystrophin is absent all the dystrophin-associated proteins are much reduced.We report here that dystrophin-associated proteins have abnormally low expression in Fukuyama-type congenital muscular dystrophy(FCMD), despite near-normal expression of dystrophin.An abnormality of dystrophin- associated proteins in sarcolemmma seems to be a common denominator in the pathological processes leading to muscle cell necrosis in three forms of severe muscular dystrophy(Duchenne,Japanese Fukuyama-type,and north Afrucab Duchenne-lie autosomal recessive).

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(click to filter results - removes previous filter) dystrophin dystrophin associated proteins muscular dystrophy muscular dystrophy,congenital muscular dystrophy,congenital,Fukuyama type

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