Journal article
Abnormal expression of dystrophin-associated proteins in Fukuyama-type congenital muscular dystrophy
The Lancet (British edition), Vol.341(8844), pp.521-522
1993
DOI: 10.1016/0140-6736(93)90279-P
PMID: 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).
Details
- Title: Subtitle
- Abnormal expression of dystrophin-associated proteins in Fukuyama-type congenital muscular dystrophy
- Creators
- K Matsumura - Howard Hughes Medical Institute and Department of Physiology and Biophysics, Univeristy of Iowa College of Medicine, Iowa City, IA 52242, U.S.AK.P Campbell - Howard Hughes Medical Institute and Department of Physiology and Biophysics, Univeristy of Iowa College of Medicine, Iowa City, IA 52242, U.S.AI Nonaka - National Institute of Neuroscience, National Centre of Neurology and Psychiatry, Tokyo, Japan
- Resource Type
- Journal article
- Publication Details
- The Lancet (British edition), Vol.341(8844), pp.521-522
- Publisher
- Elsevier Ltd
- DOI
- 10.1016/0140-6736(93)90279-P
- PMID
- 8094772
- ISSN
- 0140-6736
- eISSN
- 1474-547X
- Language
- English
- Date published
- 1993
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Iowa Neuroscience Institute
- Record Identifier
- 9984068378102771
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