Essential role of TRPV2 ion channel in the sensitivity of dystrophic muscle to eccentric contractions

Duchenne myopathy is a lethal disease due to the absence of dystrophin, a cytoskeletal protein. Muscles from dystrophin-deficient mice (mdx) typically present an exaggerated susceptibility to eccentric work characterized by an important force drop and an increased membrane permeability consecutive to repeated lengthening contractions. The present study shows that mdx muscles are largely protected from eccentric work-induced damage by overexpressing a dominant negative mutant of TRPV2 ion channel. This observation points out the role of TRPV2 channel in the physiopathology of Duchenne muscular dystrophy. (C) 2009 Federation of European Biochemical Societies. Published by Elsevier B. V. All rights reserved.

杜氏肌营养不良是一种由于抗肌萎缩蛋白（一种细胞骨架蛋白）缺失而导致的致命疾病。抗肌萎缩蛋白缺陷型小鼠（mdx）的肌肉通常对离心运动表现出过度的敏感性，其特征是在反复的拉长收缩后出现显著的力量下降以及膜通透性增加。本研究表明，通过过度表达TRPV2离子通道的显性负性突变体，mdx肌肉在很大程度上可免受离心运动诱导的损伤。这一观察结果指出了TRPV2通道在杜氏肌营养不良病理生理学中的作用。（C）2009欧洲生物化学学会联合会。由爱思唯尔B.V.出版。保留所有权利。