title: Genetic evidence for a dystrophin-glycoprotein complex (DGC) in Caenorhabditis elegans.
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-sarcoglycan and syntrophin. 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 functional analogies with the DGC exists in C. elegans.
authors.affiliation: CGMC, CNRS-UMR 5534, Université Lyon-1, 43 boulevard du 11 Novembre, 69100 Cedex, Villeurbanne, France.
authors.forename: Karine
authors.initials: K
authors.lastname: Grisoni
authors.forename: Edwige
authors.initials: E
authors.lastname: Martin
authors.forename: Kathrin
authors.initials: K
authors.lastname: Gieseler
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Mariol
authors.forename: Laurent
authors.initials: L
authors.lastname: Ségalat
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D017173:Caenorhabditis elegans / Q000235:genetics*; D003598:Cytoskeletal Proteins / Q000235:genetics; D049030:Dystroglycans; D016189:Dystrophin / Q000235:genetics*; D049029:Dystrophin-Associated Proteins; D008562:Membrane Glycoproteins / Q000235:genetics*; D008565:Membrane Proteins / Q000235:genetics; D008856:Microscopy, Fluorescence; D008969:Molecular Sequence Data; D009124:Muscle Proteins / Q000235:genetics; D009132:Muscles / Q000002:abnormalities / Q000187:drug effects / Q000378:metabolism; D034622:RNA Interference; D012330:RNA, Double-Stranded / Q000008:administration & dosage / Q000235:genetics; D016415:Sequence Alignment; D017386:Sequence Homology, Amino Acid
chemical_list: D003598:Cytoskeletal Proteins; D016189:Dystrophin; D049029:Dystrophin-Associated Proteins; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D009124:Muscle Proteins; D012330:RNA, Double-Stranded; C091824:syntrophin; D049030:Dystroglycans