α‐Dystroglycan deficiency correlates with elevated serum creatine kinase and decreased muscle contraction tension in golden retriever muscular dystrophy

FEBS Letters

Roberds, Steven L.¹  Ervasti, James M.³  Campbell, Kevin P.¹  Sharp, Nicholas J.H.²  Kornegay, Joe N.²  Anderson, Richard D.¹ 

[1] Howard Hughes Medical Institute and Department of Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, IA 52242, USA;Department of Companion Animal and Special Species Medicine, College of Veterinary Medicine, North Carolina State University, Raleigh, NC 27606, USA;Department of Physiology, University of Wisconsin Medical School, Madison, WI 53706, USA

关键词: Dystrophin;    Dystrophin—glycoprotein complex;    Dystrophin-associated protein;    Dystroglycan;    Golden retriever muscular dystrophy;   

DOI  :  10.1016/0014-5793(94)00748-9

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

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 α-dystroglycan and serum creatine kinase levels and the contraction tension measured for a given peroneus longus muscle.

【 授权许可】

Unknown