Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle

Kiichiro Matsumura, James M. Ervasti, Kay Ohlendieck, Steven D. Kahl, Kevin P. Campbell

Research output: Contribution to journalArticle

419 Citations (Scopus)

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 mice 2,6-8. 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.

Original languageEnglish (US)
Pages (from-to)588-591
Number of pages4
JournalNature
Volume360
Issue number6404
DOIs
StatePublished - Jan 1 1992

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Dystrophin-Associated Proteins
Utrophin
Inbred mdx Mouse
Sarcolemma
Duchenne Muscular Dystrophy
Muscles
Dystrophin
Neuromuscular Junction
Extracellular Matrix Proteins
Membrane Glycoproteins
Myocardium
Skeletal Muscle
Up-Regulation
Proteins
Therapeutics

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Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle. / Matsumura, Kiichiro; Ervasti, James M.; Ohlendieck, Kay; Kahl, Steven D.; Campbell, Kevin P.

In: Nature, Vol. 360, No. 6404, 01.01.1992, p. 588-591.

Research output: Contribution to journalArticle

Matsumura, Kiichiro ; Ervasti, James M. ; Ohlendieck, Kay ; Kahl, Steven D. ; Campbell, Kevin P. / Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle. In: Nature. 1992 ; Vol. 360, No. 6404. pp. 588-591.
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N2 - 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 mice 2,6-8. 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.

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