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

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 ad...

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Main Authors: Alexander Mensch, Stephan Zierz
Format: Article
Language:English
Published: MDPI AG 2020-08-01
Series:International Journal of Molecular Sciences
Subjects:
Online Access:https://www.mdpi.com/1422-0067/21/16/5830
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author Alexander Mensch
Stephan Zierz
author_facet Alexander Mensch
Stephan Zierz
author_sort Alexander Mensch
collection DOAJ
description 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.
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spelling doaj.art-3c6b9cd2024e459f8b63ace2f38bb6062023-11-20T10:06:12ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672020-08-012116583010.3390/ijms21165830Cellular Stress in the Pathogenesis of Muscular Disorders—From Cause to ConsequenceAlexander Mensch0Stephan Zierz1Department of Neurology, Martin-Luther-University of Halle-Wittenberg, 06120 Halle (Saale), GermanyDepartment of Neurology, Martin-Luther-University of Halle-Wittenberg, 06120 Halle (Saale), GermanyCellular 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.https://www.mdpi.com/1422-0067/21/16/5830myopathymuscular dystrophypathomechanismoxidative stressER-stressunfolded protein response
spellingShingle Alexander Mensch
Stephan Zierz
Cellular Stress in the Pathogenesis of Muscular Disorders—From Cause to Consequence
International Journal of Molecular Sciences
myopathy
muscular dystrophy
pathomechanism
oxidative stress
ER-stress
unfolded protein response
title Cellular Stress in the Pathogenesis of Muscular Disorders—From Cause to Consequence
title_full Cellular Stress in the Pathogenesis of Muscular Disorders—From Cause to Consequence
title_fullStr Cellular Stress in the Pathogenesis of Muscular Disorders—From Cause to Consequence
title_full_unstemmed Cellular Stress in the Pathogenesis of Muscular Disorders—From Cause to Consequence
title_short Cellular Stress in the Pathogenesis of Muscular Disorders—From Cause to Consequence
title_sort cellular stress in the pathogenesis of muscular disorders from cause to consequence
topic myopathy
muscular dystrophy
pathomechanism
oxidative stress
ER-stress
unfolded protein response
url https://www.mdpi.com/1422-0067/21/16/5830
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