Alpha-dystroglycan deficiency correlates with elevated serum creatine kinase and decreased muscle contraction tension in golden retriever muscular dystrophy.
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.
Ervasti
J M
JM
Department of Physiology, University of Wisconsin Medical School, Madison 53706.
Roberds
S L
SL
Anderson
R D
RD
Sharp
N J
NJ
Kornegay
J N
JN
Campbell
K P
KP
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*
D003598:Cytoskeletal Proteins; D016189:Dystrophin; D046911:Macromolecular Substances; D008562:Membrane Glycoproteins; D049030:Dystroglycans; D003402:Creatine Kinase