title: Alpha-dystroglycan deficiency correlates with elevated serum creatine kinase and decreased muscle contraction tension in golden retriever muscular dystrophy.
abstract: The dystrophin-glycoprotein complex was examined in dystrophin-deficient dogs with golden retriever muscular dystrophy (GRMD) using immunoblot and immunofluorescence analysis. The dystrophin-associated proteins were substantially reduced in muscle from dogs with GRMD. Interestingly, regression analysis revealed a strong correlation between the amount of alpha-dystroglycan and serum creatine kinase levels and the contraction tension measured for a given peroneus longus muscle.
authors.affiliation: Department of Physiology, University of Wisconsin Medical School, Madison 53706.
authors.forename: J M
authors.initials: JM
authors.lastname: Ervasti
authors.forename: S L
authors.initials: SL
authors.lastname: Roberds
authors.forename: R D
authors.initials: RD
authors.lastname: Anderson
authors.forename: N J
authors.initials: NJ
authors.lastname: Sharp
authors.forename: J N
authors.initials: JN
authors.lastname: Kornegay
authors.forename: K P
authors.initials: KP
authors.lastname: Campbell
mesh_terms: D000818:Animals; D003402:Creatine Kinase / Q000097:blood*; D003598:Cytoskeletal Proteins / Q000172:deficiency*; D004283:Dog Diseases / Q000503:physiopathology*; D004285:Dogs; D049030:Dystroglycans; D016189:Dystrophin / Q000172:deficiency*; D046911:Macromolecular Substances; D008562:Membrane Glycoproteins / Q000172:deficiency*; D009119:Muscle Contraction; D009137:Muscular Dystrophy, Animal / Q000503:physiopathology*
chemical_list: D003598:Cytoskeletal Proteins; D016189:Dystrophin; D046911:Macromolecular Substances; D008562:Membrane Glycoproteins; D049030:Dystroglycans; D003402:Creatine Kinase