title: Differential distribution of the members of the dystrophin glycoprotein complex in mouse retina: effect of the mdx(3Cv) mutation.
abstract: Dystrophin glycoprotein complex (DGC) assembly and function require mediation by dystrophin in skeletal muscle. The existence of such complexes and the correlation with DMD phenotypes are not yet established in the central nervous system. Here we have studied the expression of DMD gene mRNAs and proteins in retina from C57BL/6 and mdx(3Cv) mouse strains. Then we have comparatively investigated the localization of dystrophin and dystrophin-associated proteins (DAPs) in both strains to analyze the repercussion of the mdx(3Cv) mutation on the retinal distributions of alpha/beta-dystroglycan, alpha1-syntrophin, alpha-dystrobrevin, and delta/gamma-sarcoglycan. Results showed that DMD gene product deficiency affects the expression of dystroglycan assembly exclusively at the outer plexiform layer without an apparent effect on the other DAPs. We conclude that the localization of members of the DGC could be independent of the presence of the DMD gene products and/or utrophin.
authors.affiliation: Laboratoire de Physiopathologie Cellulaire et Moléculaire de la Rétine, Médicale A, INSERM EMI 99-18, CHRU, 1 Place de l'Hôpital, 67091 Strasbourg Cedex, France.
authors.forename: C
authors.initials: C
authors.lastname: Dalloz
authors.forename: T
authors.initials: T
authors.lastname: Claudepierre
authors.forename: F
authors.initials: F
authors.lastname: Rodius
authors.forename: D
authors.initials: D
authors.lastname: Mornet
authors.forename: J
authors.initials: J
authors.lastname: Sahel
authors.forename: A
authors.initials: A
authors.lastname: Rendon
mesh_terms: D000818:Animals; D002135:Calcium-Binding Proteins; D003598:Cytoskeletal Proteins / Q000235:genetics / Q000378:metabolism*; D049030:Dystroglycans; D016189:Dystrophin / Q000235:genetics / Q000378:metabolism*; D049029:Dystrophin-Associated Proteins; D015870:Gene Expression / Q000502:physiology; D008562:Membrane Glycoproteins / Q000235:genetics / Q000378:metabolism*; D008565:Membrane Proteins / Q000235:genetics / Q000378:metabolism; D051379:Mice; D008810:Mice, Inbred C57BL / Q000196:embryology / Q000235:genetics / Q000378:metabolism; D018101:Mice, Inbred mdx / Q000002:abnormalities / Q000235:genetics / Q000378:metabolism*; D009124:Muscle Proteins / Q000235:genetics / Q000378:metabolism; D020388:Muscular Dystrophy, Duchenne / Q000150:complications / Q000235:genetics* / Q000378:metabolism; D009154:Mutation / Q000235:genetics; D012333:RNA, Messenger / Q000378:metabolism; D012160:Retina / Q000002:abnormalities / Q000378:metabolism* / Q000503:physiopathology; D012164:Retinal Diseases / Q000235:genetics* / Q000378:metabolism / Q000503:physiopathology; D049031:Sarcoglycans
chemical_list: D002135:Calcium-Binding Proteins; D003598:Cytoskeletal Proteins; D016189:Dystrophin; D049029:Dystrophin-Associated Proteins; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D009124:Muscle Proteins; D012333:RNA, Messenger; D049031:Sarcoglycans; C098885:dystrobrevin; C091825:syntrophin alpha1; D049030:Dystroglycans