囊性纤维化跨膜传导调节因子氯离子通道在心脏疾病中作用研究进展  

Role of cystic fibrosis transmembrane conductance regulator chloride channel in heart diseases

作　　者：朱耀斌[1] 张雅娉[2] 李志强[1] 丁楠[1] 伊寒露 沈磊[1] 赵宇东[1] 叶赞凯 闫道乐 ZHU Yaobin;ZHANG Yaping;LI Zhiqiang;DING Nan;YI Hanlu;SHEN Lei;ZHAO Yudong;YE Zankai;YAN Daole(Department of Pediatric Cardiac Surgery,Beijing Children's Hospital Affiliated to Capital Medical University,National Children's Medical Center,Beijing 100045,China;Department of Cardiovascular Surgery,Beijing Friendship Hospital Affiliated to Capital Medical University,Beijing 100037,China)

机构地区：[1]国家儿童医学中心首都医科大学附属北京儿童医院小儿心脏外科,北京100045 [2]首都医科大学附属北京友谊医院心外科,北京100037

出　　处：《中华实用诊断与治疗杂志》2019年第12期1242-1245,共4页Journal of Chinese Practical Diagnosis and Therapy

基　　金：北京市卫生系统高层次卫生技术人才-学科骨干(2015-3-048);京市卫生系统高层次卫生技术人才-学科骨干(2015-3-051);北京市卫生系统高层次卫生技术人才-学科骨干(2014-3-043);北京市自然科学基金(7184204);北京市自然科学基金(7182042);北京市医管局培育计划(PX2016046);北京市科委首都临床特色应用研究(Z171100001017048);北京市医院管理中心儿科学科协同发展中心专项经费资助(XTYB201819)

摘　　要：细胞内外带电粒子通过心肌细胞离子通道可产生电位差,传导生物电活动、兴奋心肌细胞、使心肌产生自主电活动,引起心肌收缩。心肌细胞上存在的离子通道主要包括钠离子通道、钾离子通道、钙离子通道、氯离子通道等,参与心肌的代谢过程,是心脏疾病发生、发展的基础。氯离子是最丰富的阴离子,在细胞膜和细胞器膜上广泛存在,参与细胞增殖、凋亡、容量调节、细胞兴奋性调节等多种病理生理过程,囊性纤维化跨膜传导调节因子(cystic fibrosis transmembrane conductance regulator,CFTR)是重要的氯离子通道,CFTR突变易引起囊性纤维化疾病,主要影响消化道、呼吸道等上皮功能,关于CFTR的研究主要集中在囊性纤维化相关的疾病,近年研究发现CFTR参与心肌缺血、心律失常等心肌疾病的发病过程。本文就CFTR在心脏疾病中作用的研究进展作一综述。Intracellular and extracellular charged particles can produce potential difference through the ion channel of the myocardial cells,and can conduct bioelectrical activity,excite myocardial cells,make the heart muscle produce autonomous electrical activity,and cause myocardial contraction.The ion channels existing on the myocardial cells include sodium channels,potassium channels,calcium channels and chloride channels,all of which participate in the metabolic process of the heart muscle,and are the basis of the development of heart diseases.Chloride ion is the most abundant anion,widely existing in cell membrane and organelle membrane,and it is involved in many pathophysiological processes such as cell proliferation,apoptosis,capacity regulation,and cell excitability regulation.Cystic fibrosis transmembrane conductance regulator(CFTR)is an important chloride channel.CFTR mutations are likely to cause cystic fibrosis disease,mainly affecting the epithelial function of the digestive tract,respiratory tract,and so on.Researches of CFTR have focused on cystic fibrosis related diseases.Recent studies have suggested that CFTR is involved in the pathogenesis of myocardial ischemia and arrhythmia.This paper reviews the research progress of the role of CFTR in heart diseases.

关 键 词：心肌缺血 心律失常 囊性纤维化跨膜传导调节因子 氯离子通道 

分 类 号：R541[医药卫生—心血管疾病]