《生命科学》 2015, 27(10): 1225-1231

核孔复合物与心肌分化、增殖及心脏疾病的研究进展

冯　京1，2，徐　亮1，2，3，4*

(1 同济大学医学院病理生理学系，上海 200092；2 心律失常教育部重点实验室(同济大学)，上海 200092；3 同济大学附属东方医院心内科，上海 200120；4 同济大学医学遗传研究所，上海 200092)

摘　要：摘 要：核孔复合物(nuclear pore complexes， NPCs) 是由约30 种核孔蛋白(nucleoporins， Nups) 组成的细胞内最大的复合体。作为细胞核的“阀门”，NPCs 的主要作用是调节细胞核与细胞质间的分子转运，可参与基因表达调控等各种生理过程。心脏是维持机体新陈代谢最为重要的器官，越来越多的研究表明，NPCs 不仅参与了心脏正常生理过程如心肌分化、增殖等，也参与了许多病理过程如先天性心脏病(congenital heart disease， CHD)、房颤(atrial fibrillation， AF)、心衰(heart failure) 等。现将对NPCs 在心肌分化、增殖及心脏
    疾病等方面的进展作一综述。

Relationship between nuclear pore complexes and myocardial differentiation， proliferation and heart disease

FENG Jing1，2， XU Liang1，2，3，4*

(1 Department of Pathophysiology， School of Medicine， Tongji University， Shanghai 200092， China; 2 Key Laboratory of Arrhythmias Ministry of Education of China， Tongji University， Shanghai 200092， China; 3 Department of Cardiology， East Hospital， Tongji University School of Medicine， Shanghai 200120， China; 4 Institute of Medical Genetics， Tongji University School of Medicine， Shanghai 200065， China)

Abstract: Abstract: Nuclear pore complexes (NPCs)， composed of multiple copies of around 30 different nucleoporins (Nups)， are among the biggest proteinaceous assemblies in the cell. Residing at the nuclear envelope， they function as gatekeepers of the nucleus， performing the essential cellular role of mediating the exchange of molecules between the nucleoplasm and the cytoplasm. The heart is the most important organ for metabolism. There are compelling evidences indicating that NPCs participate in numerous physiological processes such as regulation of gene expression and are involved in multiple diseases such as congenital heart disease， atrial fibrillation and heart failure. Here we review advances of NPCs in myocardium differentiation， proliferation and heart disease.