title: Overexpression of dystrobrevin delays locomotion defects and muscle degeneration in a dystrophin-deficient Caenorhabditis elegans.
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 (dys-1) 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.
authors.affiliation: CGMC, CNRS-UMR 5534, Université Lyon 1, 43 bld du 11 Novembre, 69622, Villeurbanne cedex, France.
authors.forename: Kathrin
authors.initials: K
authors.lastname: Gieseler
authors.forename: Karine
authors.initials: K
authors.lastname: Grisoni
authors.forename: Marie Christine
authors.initials: MC
authors.lastname: Mariol
authors.forename: Laurent
authors.initials: L
authors.lastname: Ségalat
mesh_terms: D000818:Animals; D017173:Caenorhabditis elegans; D029742:Caenorhabditis elegans Proteins; D004195:Disease Models, Animal; D016189:Dystrophin / Q000235:genetics*; D015870:Gene Expression / Q000502:physiology; D008124:Locomotion / Q000235:genetics*; D009132:Muscles / Q000378:metabolism / Q000473:pathology; D020388:Muscular Dystrophy, Duchenne / Q000235:genetics; D009419:Nerve Tissue Proteins; D009479:Neuropeptides / Q000235:genetics* / Q000378:metabolism*; D010641:Phenotype
chemical_list: D029742:Caenorhabditis elegans Proteins; C479075:Dyb-1 protein, C elegans; D016189:Dystrophin; D009419:Nerve Tissue Proteins; D009479:Neuropeptides