| 摘要: |
| 心脏发挥正常功能依赖于其高度精细化的亚细胞结构。膜连接复合物(junctional membrane complexes,JMCs)是心脏正常兴奋收缩偶联(excitation contraction coupling,ECC)的关键结构。Junctophilin-2蛋白(JPH2)是公认的JMC重要结构蛋白。早期对JPH2的认识仅限于其在心肌细胞内的膜偶联功能,现在的大量研究发现JPH2在心肌细胞的T管(transverse-tubule,T-tubule)发育、心力衰竭、心肌病、心律失常的发生发展过程中也起着十分重要的作用。此综述主要归纳总结了JPH2在上述生理或病理情况中的最新研究进展,并对JPH2进一步研究方向进行了展望。 |
| 关键词: 亲联蛋白2 膜连接复合物 钙信号 心力衰竭 肥厚性心肌病 心律失常 |
| DOI：10.3724/SP.J.1008.2015.00429 |
| 投稿时间：2014-10-14修订日期：2015-01-10 |
| 基金项目:上海市申康基金(SHDC12014107). |
|
| Junctophilin-2 protein in cardiac myocytes: progress in physiological and pathophysiological functions |
| SHEN Hua1,LI Miao-ling2,HE Deng-ke1,YIN Liang1,XI Wang1,WANG Zhi-nong1* |
(1. Department of Cardiothoracic Surgery, Changzheng Hospital, Second Military Medical University, Shanghai 200003, China;
2. Department of Myocardial Electrophysiology, Luzhou Medical College, Luzhou 646000, Sichuan, China
*Corresponding author) |
| Abstract: |
| The normal cardiac function relies on highly specialized subcellular architectures. The subcellular domains junctional membrane complexes (JMCs) are essential for excitation-contraction coupling of the myocardium. Junctophilin-2 (JPH2) has been widely recognized as the crucial structural protein involved in JMCs. Initial studies limited the role of JPH2 to anchoring junctional sarcoplasmic reticulum (SR) and transverse-tubule (T-tubule) membrane invaginations. Recently, researchers have found an expanded role of JPH2 in the development of postnatal T-tubule in mammals, progression of disease in failing hearts, hypertrophic cardiomyopathy and arrhythmias. In this review we summarized the role of JPH2 in the above physiological or pathophysiological processes and discussed the perspective in future investigation. |
| Key words: junctophilin 2 junctional membrane complex calcium signaling heart failure hypertrophic cardiomyopathy arrhythmia |
