Henderson Christine A, Gomez Christopher G, Novak Stefanie M, Mi-Mi Lei, Gregorio Carol C
Department of Cellular and Molecular Medicine, The University of Arizona, Tucson, Arizona, USA.
Sarver Molecular Cardiovascular Research Program, The University of Arizona, Tucson, Arizona, USA.
Compr Physiol. 2017 Jun 18;7(3):891-944. doi: 10.1002/cphy.c160033.
Cardiac and skeletal striated muscles are intricately designed machines responsible for muscle contraction. Coordination of the basic contractile unit, the sarcomere, and the complex cytoskeletal networks are critical for contractile activity. The sarcomere is comprised of precisely organized individual filament systems that include thin (actin), thick (myosin), titin, and nebulin. Connecting the sarcomere to other organelles (e.g., mitochondria and nucleus) and serving as the scaffold to maintain cellular integrity are the intermediate filaments. The costamere, on the other hand, tethers the sarcomere to the cell membrane. Unique structures like the intercalated disc in cardiac muscle and the myotendinous junction in skeletal muscle help synchronize and transmit force. Intense investigation has been done on many of the proteins that make up these cytoskeletal assemblies. Yet the details of their function and how they interconnect have just started to be elucidated. A vast number of human myopathies are contributed to mutations in muscle proteins; thus understanding their basic function provides a mechanistic understanding of muscle disorders. In this review, we highlight the components of striated muscle with respect to their interactions, signaling pathways, functions, and connections to disease. © 2017 American Physiological Society. Compr Physiol 7:891-944, 2017.
心肌和骨骼肌是负责肌肉收缩的精密设计的机器。基本收缩单位肌节以及复杂的细胞骨架网络的协调对于收缩活动至关重要。肌节由精确组织的单个细丝系统组成,包括细肌丝(肌动蛋白)、粗肌丝(肌球蛋白)、肌联蛋白和伴肌动蛋白。中间丝将肌节与其他细胞器(如线粒体和细胞核)相连,并作为维持细胞完整性的支架。另一方面,肌附着点将肌节与细胞膜相连。像心肌中的闰盘和骨骼肌中的肌腱连接这样的独特结构有助于同步和传递力量。人们已经对构成这些细胞骨架组件的许多蛋白质进行了深入研究。然而,它们的功能细节以及它们如何相互连接才刚刚开始被阐明。大量人类肌病是由肌肉蛋白突变引起的;因此,了解它们的基本功能有助于从机制上理解肌肉疾病。在这篇综述中,我们重点介绍横纹肌的组成部分,包括它们的相互作用、信号通路、功能以及与疾病的联系。© 2017美国生理学会。《综合生理学》7:891 - 944, 2017。