title: 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.
authors.affiliation: Department of Biochemistry and Molecular Biology, School of Medicine, University of Maryland, 108 N Greene Street, Baltimore, MD 21201, USA.
authors.forename: Li-Yen R
authors.initials: LY
authors.lastname: Hu
authors.affiliation: Department of Biochemistry and Molecular Biology, School of Medicine, University of Maryland, 108 N Greene Street, Baltimore, MD 21201, USA.
authors.forename: Maegen A
authors.initials: MA
authors.lastname: Ackermann
authors.affiliation: Department of Biochemistry and Molecular Biology, School of Medicine, University of Maryland, 108 N Greene Street, Baltimore, MD 21201, USA.
authors.forename: Aikaterini
authors.initials: A
authors.lastname: Kontrogianni-Konstantopoulos
mesh_terms: D003599:Cytoskeleton / Q000378:metabolism*; D006801:Humans; D009119:Muscle Contraction; D009124:Muscle Proteins / Q000737:chemistry* / Q000378:metabolism; D009206:Myocardium / Q000378:metabolism*; D012518:Sarcomeres / Q000737:chemistry / Q000378:metabolism*; D013314:Stress, Mechanical
chemical_list: D009124:Muscle Proteins