CGC Bibliography Paper 5290

Overexpression of dystrobrevin delays locomotion defects and muscle degeneration in a dystrophin-deficient Caenorhabditis elegans.

Gieseler K, Grisoni K, Mariol MC, Segalat L

Medline:

Citation:

Neuromuscular Disorders 12: 371-377 2002

Type:

ARTICLE

Genes:

dyb-1 dys-1 hlh-1

Abstract:

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.