Abnormal expression of dystrophin- associated proteins in Fukuyama-type congenital muscular dystrophy.
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).[1]References
- Abnormal expression of dystrophin-associated proteins in Fukuyama-type congenital muscular dystrophy. Matsumura, K., Nonaka, I., Campbell, K.P. Lancet (1993) [Pubmed]
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