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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MDPI AG
2020-08-01
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Series: | International Journal of Molecular Sciences |
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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. |
first_indexed | 2024-03-10T17:28:13Z |
format | Article |
id | doaj.art-3c6b9cd2024e459f8b63ace2f38bb606 |
institution | Directory Open Access Journal |
issn | 1661-6596 1422-0067 |
language | English |
last_indexed | 2024-03-10T17:28:13Z |
publishDate | 2020-08-01 |
publisher | MDPI AG |
record_format | Article |
series | International Journal of Molecular Sciences |
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 |
work_keys_str_mv | AT alexandermensch cellularstressinthepathogenesisofmusculardisordersfromcausetoconsequence AT stephanzierz cellularstressinthepathogenesisofmusculardisordersfromcausetoconsequence |