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  • Title: Abnormal expression of dystrophin-associated proteins in Fukuyama-type congenital muscular dystrophy.
    Author: Matsumura K, Nonaka I, Campbell KP.
    Journal: Lancet; 1993 Feb 27; 341(8844):521-2. PubMed ID: 8094772.
    Abstract:
    The absence of dystrophin causes Duchenne muscular dystrophy. Dystrophin is associated with a large complex of sarcolemmal glycoproteins which provides 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 the sarcolemma 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 African Duchenne-like autosomal recessive).
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