Distal mdx muscle groups exhibiting up-regulation of utrophin and rescue of dystrophin-associated glycoproteins exemplify a protected phenotype in muscular dystrophy


Dowling, Paul and Culligan, Kevin and Ohlendieck, Kay (2002) Distal mdx muscle groups exhibiting up-regulation of utrophin and rescue of dystrophin-associated glycoproteins exemplify a protected phenotype in muscular dystrophy. Naturwissenschaften, 89 (2). pp. 75-78. ISSN 0028-1042

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Abstract

Unique unaffected skeletal muscle fibres, unlike necrotic torso and limb muscles, may pave the way for a more detailed understanding of the molecular pathogenesis of inherited neuromuscular disorders and help to develop new treatment strategies for muscular dystrophies. The sparing of extraocular muscle in Duchenne muscular dystrophy is mostly attributed to the special protective properties of extremely fast-twitching smalldiameter fibres, but here we show that distal muscles also represent a particular phenotype that is more resistant to necrosis. Immunoblot analysis of membranes isolated from the well established dystrophic animal model mdx shows that, in contrast to dystrophic limb muscles, the toe musculature exhibits an up-regulation of the autosomal dystrophin homologue utrophin and a concomitant rescue of dystrophin-associated glycoproteins. Thus distal mdx muscle groups provide a cellular system that naturally avoids myofibre degeneration which might be useful in the search for naturally occurring compensatory mechanisms in inherited skeletal muscle diseases.

Item Type: Article
Keywords: Distal mdx; muscle groups; up-regulation; utrophin; dystrophin-associated glycoproteins; protected phenotype; muscular dystrophy;
Academic Unit: Faculty of Science and Engineering > Biology
Item ID: 7341
Identification Number: 10.1007/s00114-001-0289-4
Depositing User: Paul Dowling
Date Deposited: 15 Aug 2016 16:05
Journal or Publication Title: Naturwissenschaften
Publisher: Springer
Refereed: Yes
Funders: Health Research Board (HRB), Enterprise Ireland (EI), Royal Society, London, Royal Irish Academy
URI:

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