肌肉中钙蛋白酶-3的研究进展  

Research progress of CAPN3 in muscle

作　　者：尹淑琴[1] 宋彩丽 张晓红 曹靖[2] 常泓 霍乃蕊[4] YIN Shuqin;SONG Caili;ZHANG Xiaohong;CAO Jing;CHANG Hong;HUO Nairui(College of Life Sciences,Shanxi Agricultural University,Taigu 030801,China;Department of Animal Husbandry and Veterinary,Beijing Vocational College of Agriculture,Beijing 102442,China;journal of Shanxi Agricultural University,Taigu 030801,China;College of Animal Science and Technplogy,Shanxi Agricultural University,Taigu 030801,China)

机构地区：[1]山西农业大学生命科学学院,太谷030801 [2]北京农业职业学院畜牧兽医系,北京102442 [3]山西农业大学期刊社,太谷030801 [4]山西农业大学动物科技学院,太谷030801

出　　处：《生命的化学》2018年第5期681-688,共8页Chemistry of Life

基　　金：国家自然科学基金项目(31201347);山西省科技重点研发项目(2015-TN-10-2)

摘　　要：钙蛋白酶(calpain/CAPN)是参与一系列生物活动的钙调节蛋白酶。在细胞内,calpain通过对底物限制性的水解来调节蛋白质的各种功能。钙蛋白酶-3(CAPN3),也称p94或calpain-3,是一种骨骼肌特异性的蛋白酶,具有其他calpain甚至是其它蛋白酶所不具有的特性:CAPN3能够快速、彻底地自动降解,并且可以通过分子内互补作用(intermolecular complementation, iMOC)重新获得活性;CAPN3是细胞内迄今为止唯一的依赖于Na+激活的酶等。此外,基因突变导致CAPN3蛋白缺失会引起肢带型肌营养不良2A症(limb-girdle muscular dystrophy type 2A, LGMD2A)。本文综述了CAPN3从发现到目前的研究进展,同时对该领域的研究趋势进行了讨论和展望。Calpain/CAPN is a calmodulating protease that is involved in a series of biological activities. Calpains process substrates by limited proteolysis to modulate various protein functions in the cell. CAPN3, previously called p94 or calpain-3, is a skeletal muscle specific protease that has unique features that are not found in any of the other human calpains, or even in other proteases. For instance, CAPN3 undergoes extremely rapid and exhaustive autodegradation, and it has the ability to regain protease function after its autolytic dissociation through a process known as intermolecular complementation （iMOC）. CAPN3 is also the first （and so far, the only） intracellular enzyme found to depend on Na＋ for its activation. In addition, gene mutations causing CAPN3 defectsare responsible for limb-girdle muscular dystrophy type 2A （LGMD2A）. In this review, we describe significant findings about CAPN3 from its discovery to the present, and the future research interests and prospective in this field are discussed.

关 键 词：钙蛋白酶 结构-活性的关系 自溶 肢带型肌营养不良2A症 

分 类 号：Q55[生物学—生物化学]