Insights into myosin regulatory and essential light chains: a focus on their roles in cardiac and skeletal muscle function, development and disease.

IF 1.8 3区生物学 Q4 CELL BIOLOGY

Journal of Muscle Research and Cell Motility Pub Date : 2020-12-01 Epub Date: 2019-05-27 DOI:10.1007/s10974-019-09517-x

Yoel H Sitbon, Sunil Yadav, Katarzyna Kazmierczak, Danuta Szczesna-Cordary

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引用次数: 0

Abstract

The activity of cardiac and skeletal muscles depends upon the ATP-coupled actin-myosin interactions to execute the power stroke and muscle contraction. The goal of this review article is to provide insight into the function of myosin II, the molecular motor of the heart and skeletal muscles, with a special focus on the role of myosin II light chain (MLC) components. Specifically, we focus on the involvement of myosin regulatory (RLC) and essential (ELC) light chains in striated muscle development, isoform appearance and their function in normal and diseased muscle. We review the consequences of isoform switching and knockout of specific MLC isoforms on cardiac and skeletal muscle function in various animal models. Finally, we discuss how dysregulation of specific RLC/ELC isoforms can lead to cardiac and skeletal muscle diseases and summarize the effects of most studied mutations leading to cardiac or skeletal myopathies.

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深入了解肌球蛋白调节链和基本轻链：重点关注它们在心肌和骨骼肌功能、发育和疾病中的作用。

心脏和骨骼肌的活动依赖于 ATP 耦合的肌动蛋白-肌球蛋白相互作用来执行动力冲程和肌肉收缩。这篇综述文章旨在深入探讨肌球蛋白 II（心脏和骨骼肌的分子马达）的功能，特别关注肌球蛋白 II 轻链（MLC）成分的作用。具体来说，我们重点研究肌球蛋白调节轻链（RLC）和基本轻链（ELC）在横纹肌发育中的参与、同工酶的出现及其在正常和患病肌肉中的功能。我们回顾了在各种动物模型中，同工酶同工酶的转换和特定 MLC 同工酶的敲除对心肌和骨骼肌功能的影响。最后，我们讨论了特定 RLC/ELC 同工酶的失调如何导致心肌和骨骼肌疾病，并总结了导致心肌或骨骼肌疾病的大多数研究突变的影响。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of Muscle Research and Cell Motility 生物-细胞生物学

CiteScore

6.20

自引率

0.00%

发文量

审稿时长

>12 weeks

期刊介绍： The Journal of Muscle Research and Cell Motility has as its main aim the publication of original research which bears on either the excitation and contraction of muscle, the analysis of any one of the processes involved therein, the processes underlying contractility and motility of animal and plant cells, the toxicology and pharmacology related to contractility, or the formation, dynamics and turnover of contractile structures in muscle and non-muscle cells. Studies describing the impact of pathogenic mutations in genes encoding components of contractile structures in humans or animals are welcome, provided they offer mechanistic insight into the disease process or the underlying gene function. The policy of the Journal is to encourage any form of novel practical study whatever its specialist interest, as long as it falls within this broad field. Theoretical essays are welcome provided that they are concise and suggest practical ways in which they may be tested. Manuscripts reporting new mutations in known disease genes without validation and mechanistic insight will not be considered. It is the policy of the journal that cells lines, hybridomas and DNA clones should be made available by the developers to any qualified investigator. Submission of a manuscript for publication constitutes an agreement of the authors to abide by this principle.