Department of Neurology, Martin-Luther-University of Halle-Wittenberg, 06120 Halle (Saale), Germany.
Int J Mol Sci. 2020 Aug 13;21(16):5830. doi: 10.3390/ijms21165830.
Cellular stress has been considered a relevant pathogenetic factor in a variety of human diseases. Due to its primary functions by means of contractility, metabolism, and protein synthesis, the muscle cell is faced with continuous changes of cellular homeostasis that require rapid and coordinated adaptive mechanisms. Hence, a prone susceptibility to cellular stress in muscle is immanent. However, studies focusing on the cellular stress response in muscular disorders are limited. While in recent years there have been emerging indications regarding a relevant role of cellular stress in the pathophysiology of several muscular disorders, the underlying mechanisms are to a great extent incompletely understood. This review aimed to summarize the available evidence regarding a deregulation of the cellular stress response in individual muscle diseases. Potential mechanisms, as well as involved pathways are critically discussed, and respective disease models are addressed. Furthermore, relevant therapeutic approaches that aim to abrogate defects of cellular stress response in muscular disorders are outlined.
细胞应激被认为是多种人类疾病的相关发病因素。由于肌肉细胞通过收缩、代谢和蛋白质合成发挥主要功能，因此它面临着持续的细胞内稳态变化，需要快速和协调的适应机制。因此，肌肉对细胞应激的易感性是内在的。然而，目前针对肌肉疾病中细胞应激反应的研究还很有限。虽然近年来有越来越多的迹象表明细胞应激在几种肌肉疾病的病理生理学中起重要作用，但其中的机制在很大程度上仍未被完全理解。本综述旨在总结关于个体肌肉疾病中细胞应激反应失调的现有证据。本文批判性地讨论了潜在的机制和相关途径，并探讨了相应的疾病模型。此外，还概述了旨在消除肌肉疾病中细胞应激反应缺陷的相关治疗方法。
