Cellular Stress in the Pathogenesis of Muscular Disorders—From Cause to Consequence

International Journal of Molecular Sciences

卷:21

Alexander Mensch¹  Stephan Zierz¹ 

[1] Department of Neurology, Martin-Luther-University of Halle-Wittenberg, 06120 Halle (Saale), Germany;

关键词: myopathy;    muscular dystrophy;    pathomechanism;    oxidative stress;    ER-stress;    unfolded protein response;   

DOI  :  10.3390/ijms21165830

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown