肌营养不良中肌球蛋白重链亚型的分布：对疾病病理学的见解
Distribution of myosin heavy chain isoforms in muscular dystrophy: insights into disease pathology.
作者信息
Beedle Aaron M
机构信息
Department of Pharmaceutical and Biomedical Sciences, University of Georgia College of Pharmacy, Athens, GA 30602 USA.
出版信息
Musculoskelet Regen. 2016;2. Epub 2016 Jul 5.
Abstract
Myosin heavy chain isoforms are an important component defining fiber type specific properties in skeletal muscle, such as oxidative versus glycolytic metabolism, rate of contraction, and fatigability. While the molecular mechanisms that underlie specification of the different fiber types are becoming clearer, how this programming becomes disrupted in muscular dystrophy and the functional consequences of fiber type changes in disease are not fully resolved. Fiber type changes in disease, with specific focus on muscular dystrophies caused by defects in the dystrophin glycoprotein complex, are discussed.
摘要
肌球蛋白重链异构体是决定骨骼肌纤维类型特异性属性的重要组成部分，如氧化代谢与糖酵解代谢、收缩速率和疲劳性。虽然不同纤维类型特化背后的分子机制正变得越来越清晰，但在肌肉营养不良中这种编程是如何被破坏的，以及疾病中纤维类型变化的功能后果尚未完全解决。本文讨论了疾病中的纤维类型变化，特别关注由肌营养不良蛋白糖蛋白复合物缺陷引起的肌肉营养不良。
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