Journal article
Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle
Nature (London), Vol.360(6404), pp.588-591
12/1992
DOI: 10.1038/360588a0
PMID: 1461282
Abstract
DYSTROPHIN is associated with a complex of muscle membrane (sarcolemmal) glycoproteins that provide a linkage to the extracellular matrix protein, laminin1–8. The absence of dystrophin leads to a dramatic reduction of the dystrophin-associated proteins (156DAG, 59DAP, 50DAG, 43DAG and 35DAG) in the sarcolemma of patients with Duchenne muscular dystrophy and mdx mice2,6–8. Here we demonstrate that dystrophin-related protein (DRP, utrophin), an autosomal homologue of dystrophin9,17, is associated with an identical or antigenically similar complex of sarcolemmal proteins and that DRP and the dystrophin/DRP-associated proteins colocalize to the neuromuscular junction in Duchenne muscular dystrophy and mdx muscle. The DRP and dystrophin/DRP-associated proteins are found throughout the sarcolemma in small-calibre skeletal muscles and cardiac muscle of adult mdx mice. Because these muscles show minimal pathological changes18–20, our results could provide a basis for the upregulation of DRP as a potential therapeutic approach.
Details
- Title: Subtitle
- Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle
- Creators
- Kiichiro MatsumuraJames M ErvastiKay OhlendieckSteven D KahlKevin P Campbell
- Resource Type
- Journal article
- Publication Details
- Nature (London), Vol.360(6404), pp.588-591
- DOI
- 10.1038/360588a0
- PMID
- 1461282
- ISSN
- 0028-0836
- eISSN
- 1476-4687
- Language
- English
- Date published
- 12/1992
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Iowa Neuroscience Institute
- Record Identifier
- 9984020735802771
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