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肌肉萎缩症中的膜损伤与修复。

Membrane Injury and Repair in the Muscular Dystrophies.

机构信息

Institute for Neuroscience and Muscle Research, Kids Research Institute, The Children's Hospital at Westmead, Sydney, New South Wales, Australia Discipline of Paediatrics and Child Health, Faculty of Medicine, University of Sydney, New South Wales, Australia

School of Medical Sciences, University of New South Wales, Sydney, New South Wales, Australia.

出版信息

Neuroscientist. 2015 Dec;21(6):653-68. doi: 10.1177/1073858414558336. Epub 2014 Nov 18.

Abstract

Muscle cells have an elaborate plasma membrane and t-tubule system that has been evolutionarily refined to maximize electrical conductivity for synchronous muscle contraction. However, this elaborate plasma membrane network has intrinsic vulnerabilities to stretch-induced membrane injury, and thus requires ongoing maintenance and repair. Herein we discuss the types of membrane injuries encountered by myofibers in healthy muscle and in muscular dystrophy. We review the different mechanisms by which muscle fibers in patients with muscular dystrophy are rendered more susceptible to injury, and we summarize the latest developments in our understanding of how the muscular dystrophy protein dysferlin mediates satellite-cell independent membrane repair.

摘要

肌细胞具有精细的质膜和 T 管系统,这一系统经过进化的完善,最大限度地提高了肌肉收缩的导电性。然而,这种精细的质膜网络对拉伸引起的膜损伤具有内在的脆弱性,因此需要持续的维护和修复。本文讨论了健康肌肉和肌肉疾病中肌纤维所遇到的膜损伤类型。我们回顾了肌肉疾病患者的肌纤维更容易受到损伤的不同机制,并总结了我们对肌营养不良蛋白 dysferlin 如何介导卫星细胞非依赖性膜修复的最新理解。

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