Ahmad Khurshid, Shaikh Sibhghatulla, Ahmad Syed Sayeed, Lee Eun Ju, Choi Inho
Department of Medical Biotechnology, Yeungnam University, Gyeongsan, South Korea.
Front Pharmacol. 2020 Feb 28;11:142. doi: 10.3389/fphar.2020.00142. eCollection 2020.
Skeletal muscle (SM) comprises around 40% of total body weight and is among the most important plastic tissues, as it supports skeletal development, controls body temperature, and manages glucose levels. Extracellular matrix (ECM) maintains the integrity of SM, enables biochemical signaling, provides structural support, and plays a vital role during myogenesis. Several human diseases are coupled with dysfunctions of the ECM, and several ECM components are involved in disease pathologies that affect almost all organ systems. Thus, mutations in ECM genes that encode proteins and their transmembrane receptors can result in diverse SM diseases, a large proportion of which are types of fibrosis and muscular dystrophy. In this review, we present major ECM components of SMs related to muscle-associated diseases, and discuss two major ECM myopathies, namely, collagen myopathy and laminin myopathies, and their therapeutic managements. A comprehensive understanding of the mechanisms underlying these ECM-related myopathies would undoubtedly aid the discovery of novel treatments for these devastating diseases.
骨骼肌（SM）约占体重的40%，是最重要的可塑性组织之一，因为它支持骨骼发育、控制体温并调节血糖水平。细胞外基质（ECM）维持骨骼肌的完整性，实现生化信号传导，提供结构支持，并在肌生成过程中发挥至关重要的作用。几种人类疾病与细胞外基质功能障碍相关，并且几种细胞外基质成分参与了几乎影响所有器官系统的疾病病理过程。因此，编码蛋白质及其跨膜受体的细胞外基质基因突变可导致多种骨骼肌疾病，其中很大一部分是纤维化和肌肉萎缩症类型。在本综述中，我们介绍了与肌肉相关疾病相关的骨骼肌主要细胞外基质成分，并讨论了两种主要的细胞外基质肌病，即胶原肌病和层粘连蛋白肌病及其治疗管理。全面了解这些与细胞外基质相关的肌病的潜在机制无疑将有助于发现针对这些毁灭性疾病的新疗法。
