中文摘要：

小泛素样修饰蛋白（small ubiquitin-related modifiers,SUMO）是类泛素蛋白家族中的一员。SUMO化是一种蛋白翻译后修饰,借助一系列酶促反应与底物发生可逆的共价结合,参与并调节许多细胞内生理活动,包括细胞周期,DNA复制与修复,转录调节,染色质重建,蛋白质间相互作用,亚细胞结构定位,促进蛋白酶体降解等,这一系列的生理作用被证实与神经系统疾病,肿瘤的发展与转移等多种疾病密切相关。近年来,SUMO化在心血管系统中的作用倍受关注,本文主要阐述SUMO化在心脏结构发育过程及其对先天性心脏病影响的研究进展。

英文摘要：

Small ubiquitin-related modifiers（SUMO）is one of ubiquitin-like proteins.Sumoylation is a posttranslational modification process in which SUMO proteins are covalently and reversibly conjugated to their targets via enzymatic cascade reactions.Sumoylation of protein plays an important role in a number of biological activities such as cell cycle progression,DNA replication/repair,transcription modification,changes in subcellular localization alteration of protein-protein interaction,promoting proteasomal degradation.A lot of evidences show that SUMOs involved in human pathogenesis such as cancer development and metastasis,neurodegenerative disorders and craniofacial defects.The role of the SUMO pathway in regulating cardiogenic gene activity,development and/or disorders is just emerging.This review based on the advanced researches tries to explain the potential links between SUMO and congenital heart diseases.