Journal
NEUROMUSCULAR DISORDERS
Volume 12, Issue 4, Pages 371-377Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/S0960-8966(01)00330-3
Keywords
dystrobrevin; dystrophin; Duchenne muscular dystrophy; nematode
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Duchenne muscular dystrophy is one of the most common neuromuscular diseases. It is caused by mutations in the dystrophin gene. Dystrobrevins are dystrophin-associated proteins potentially involved in signal transduction. The nematode Caenorhabditis elegans possesses one dystrophin-like and one dystrobrevin-like (dyb-1) gene. Mutations of dyb-1 and dys-1 lead to similar phenotypes, comprising hyperactivity and a tendency to hypercontract, which suggest that these proteins may participate in a common function. We show here that overexpression of the Dyb-1 protein delays the onset of the myopathy observed in the C. elegans double Mutant (dys-1; hlh-1 mutations). This finding indicates that, in C. elegans, ( 1) the absence of dystrophin can be partly compensated for by extra doses of dystrobrevin, and (2) dystrobrevin is partly functional in absence of dystrophin. (C) 2002 Elsevier Science B.V. All rights reserved.
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