CGC Bibliography Paper 5519

Genetic evidence for a dystrophin-glycoprotein complex (DGC) in Caenorhabditis elegans.

Grisoni K, Martin E, Gieseler K, Mariol MC, Segalat L

Medline:

12234669

Citation:

Gene 294: 77-86 2002

Type:

ARTICLE

Genes:

dys-1 hlh-1

Abstract:

Dystrophin is the product of the gene mutated in Duchenne muscular dystrophy (DMD). Neither the function of dystrophin nor the physiopathology of the disease have been clearly established so far. In mammals, the dystrophin-glycoprotein complex (DGC) includes dystrophin, as well as transmembrane and cytoplasmic proteins. Since Caenorhabditis elegans possesses a dystrophin-like gene (dys-1), we investigated whether homologues of the DGC members could also be found in the C. elegans genome. Conserved homologues were found for dystroglycan, delta/gamma-sarcoglyean and syntrophim. Divergent but related proteins were found for alpha- and beta-sarcoglycans. No sarcospan counterpart was found. The expression of the conserved homologues was inactivated using the RNA interference technique. Phenotypes similar to that of dys-1 were obtained, both in the wild-type background and in combination with other mutations. These results strongly suggest that a protein complex comprising