肌肉萎缩相关蛋白Syncoilin的研究进展  

Studying on Progressing of Amyotrophy-Related Protein Syncoilin

作　　者：张辰艳[1] 沈何放[1] 呼延霆[1] 尹大川[1] 

机构地区：[1]西北工业大学生命学院空间生物实验模拟技术国防重点学科实验室,陕西西安710072

出　　处：《现代生物医学进展》2013年第18期3587-3590,共4页Progress in Modern Biomedicine

基　　金：国家自然科学基金项目(31170816;11202167);中国博士后基金面上项目项目(2012M512029);陕西省自然科学基金项目(2012JQ3009)

摘　　要：肌肉萎缩是一种严重危害人类健康的疾病。研究与肌肉萎缩相关的重要生理蛋白的结构和功能,将有利于从分子和细胞层面阐明肌肉萎缩致病的机理,是预防和治疗肌肉萎缩的重要途径。Syncoilin是2001年发现的一种新的Ⅲ型中间纤维,与其它中间纤维蛋白不同,syncoilin在体内以单体形式存在。它是肌肉萎缩蛋白复合物的成员,直接连接肌萎缩关联蛋白复合物成员之一dystrobrevin和中间纤维desmin,以此将基底膜和细胞骨架组成一个整体,该体系的完整性是肌肉伸长和收缩所必须的,该体系被破坏会影响肌肉运动中侧向力的传导,进而诱发肌肉萎缩的发生。同时,syncoilin参与肌纤维再生,其表达量的下调会影响肌纤维的重建。Syncoilin表达的紊乱,将会导致肌营养不良症、desmin相关肌病和肌肉萎缩侧索硬化症等疾病的发生。本文就近年来关于syncoilin的分子结构、组织分布、生理功能、参与的信号通路及相关疾病等方面的研究进行了综述,旨在为深入理解syncoilin参与的肌肉萎缩发生的分子机制,预防和治疗肌肉萎缩提供积极的参考。Muscular dystrophy is one of diseases greatly threaten human health.Studying the structure and function of some important amyotrophy-related proteins at the molecular and cellular level is not only benefit for understanding mechanisms of muscular dystrophy,but also important for preventing and curing such diseases.After been found in 2001,syncoilin is classified as type Ⅲ intermediate filament protein,it is monomer in vivo which is significantly different from other intermediate filament proteins.Syncoilin is one of proteins which belong to dystrophin-associated protein complex.It links dystrobrevin which belongs to dystrophin-associated protein complex to desmin,and furthermore,the integrity of this triad is critically indispensable to muscular contraction and expansion.Lateral force conduction of muscular movement is impeded after disturbing of this triad,and further induces muscular dystrophy.Syncoilin is involved in regeneration of myofiber,its down-regulation affects rebuilding of myofiber.The abnormality of syncoilin expression leads to several diseases,including muscular dystrophy,desmin-related myopathy and amyotrophic lateral sclerosis.In this paper,syncoilin＇s molecular structure,tissue distributions,physiological functions,signal pathways and related diseases will be summarized,which will be helpful to understand molecular mechanism of syncoilin-related muscular dystrophy,and gives light on preventing and curing such diseases.

关 键 词：肌肉萎缩 Syncoilin 肌萎缩关联复合物 中间纤维 分子机制 

分 类 号：Q257[生物学—细胞生物学]