上海交通大学学报（医学版）

上海交通大学学报（医学版） ›› 2018, Vol. 38 ›› Issue (4): 361-.doi: 10.3969/j.issn.1674-8115.2018.04.001

• 专栏·中国卓越国际论文述评 • 上一篇    下一篇

Wdpcp基因调控心脏冠状动脉发育的机制

刘向阳 1，CAI Chen-leng2   

  1. 1. 上海市小儿先天性心脏病研究所，上海交通大学医学院儿科转化医学研究所，上海儿童医学中心，上海200120；2. Riley Heart Research Center, Herman Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, IN 46202, USA
  • 出版日期:2018-04-28 发布日期:2018-05-03
  • 通讯作者: CAI Chen-leng，电子信箱：chenleng@iu.edu。
  • 作者简介:刘向阳（1989—），女，博士生；电子信箱：liuxy2008nian@sjtu.edu.cn。
  • 基金资助:
    国家科技部重大研究计划（2013CB945302）；国家自然科学基金（31371465，31771612）；上海市教育委员会高峰高原学科建设计划（20171925）；上海市卫生和计划生育委员会基金（XBR2015）；

Mechanism of Wdpcp in regulating coronary artery development

LIU Xiang-yang1, CAI Chen-leng2   

  1. 1. Shanghai Pediatric Congenital Heart Disease Institute, Pediatric Translational Medicine Institute, Shanghai Jiao Tong University School of Medicine, Shanghai Children’s Medical Center, Shanghai 200120;2. Riley Heart Research Center, Herman Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, IN46202, USA
  • Online:2018-04-28 Published:2018-05-03
  • Supported by:
    Key Program of the Ministry of Science and Technology, 2013CB945302;National Natural Science Foundation of China, 31371465, 31771612; Shanghai Municipal Education Commission—Gaofeng Clinical Medicine Support, 20171925;Shanghai Municipal Commission of Health and Family Planning Foundation, XBR2015

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摘要： Wdpcp作为平面细胞极性的效应基因，在胚胎时期主要通过影响细胞运动和初级纤毛生长参与多种器官和组织的发生和发育过程。WDPCP突变与临床上Bardet-Biedl综合征和Meckel-Gruber综合征相关。该文主要揭示了Wdpcp基因调节小鼠冠状动脉发育的新功能和机制，丰富了该基因的致病类型，为临床上诊断冠状动脉疾病的发病原因提供了重要的遗传学依据。

关键词: Wdpcp基因, 冠状动脉发育, 心外膜来源细胞, 上皮-间充质转化

Abstract:

Wdpcp, a planar cell polarity (PCP) effector gene, plays essential roles in organogenesis during embryonic development through regulating collective cell movement and ciliogenesis. WDPCP mutations are associated with Bardet–Biedl syndrome and Meckel–Gruber syndrome in human. This study revealed a novel role of Wdpcp in regulating coronary artery development in mouse, demonstrating a new pathogenic phenotype associated with mutations of this gene in human. Results of this study may help us to understand the etiology of coronary artery disease.

Key words: Wdpcp gene, coronary artery development, epicardial derived cell, epithelial-mesenchymal transition

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