Table of Contents Author Guidelines Submit a Manuscript
BioMed Research International
Volume 2015, Article ID 714197, 25 pages
http://dx.doi.org/10.1155/2015/714197
Review Article

The Sarcomeric M-Region: A Molecular Command Center for Diverse Cellular Processes

Department of Biochemistry and Molecular Biology, School of Medicine, University of Maryland, 108 N Greene Street, Baltimore, MD 21201, USA

Received 17 November 2014; Accepted 8 February 2015

Academic Editor: Oleg S. Matusovsky

Copyright © 2015 Li-Yen R. Hu et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.