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The sarcomeric M-region: a molecular command center for diverse cellular processes.


ABSTRACT: The sarcomeric M-region anchors thick filaments and withstands the mechanical stress of contractions by deformation, thus enabling distribution of physiological forces along the length of thick filaments. While the role of the M-region in supporting myofibrillar structure and contractility is well established, its role in mediating additional cellular processes has only recently started to emerge. As such, M-region is the hub of key protein players contributing to cytoskeletal remodeling, signal transduction, mechanosensing, metabolism, and proteasomal degradation. Mutations in genes encoding M-region related proteins lead to development of severe and lethal cardiac and skeletal myopathies affecting mankind. Herein, we describe the main cellular processes taking place at the M-region, other than thick filament assembly, and discuss human myopathies associated with mutant or truncated M-region proteins.

SUBMITTER: Hu LY 

PROVIDER: S-EPMC4413555 | biostudies-literature | 2015

REPOSITORIES: biostudies-literature

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The sarcomeric M-region: a molecular command center for diverse cellular processes.

Hu Li-Yen R LY   Ackermann Maegen A MA   Kontrogianni-Konstantopoulos Aikaterini A  

BioMed research international 20150415


The sarcomeric M-region anchors thick filaments and withstands the mechanical stress of contractions by deformation, thus enabling distribution of physiological forces along the length of thick filaments. While the role of the M-region in supporting myofibrillar structure and contractility is well established, its role in mediating additional cellular processes has only recently started to emerge. As such, M-region is the hub of key protein players contributing to cytoskeletal remodeling, signal  ...[more]

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