Homeobox protein Hop functions in the adult cardiac conduction system

被引:62
作者
Ismat, FA
Zhang, MZ
Kook, H
Huang, B
Zhou, R
Ferrari, VA
Epstein, JA
Patel, VV
机构
[1] Childrens Hosp Philadelphia, Dept Pediat, Div Cardiol, Philadelphia, PA 19104 USA
[2] Univ Penn, Div Cardiovasc Med, Philadelphia, PA 19104 USA
[3] Univ Penn, Dept Radiol, Philadelphia, PA 19104 USA
[4] Chonnam Natl Univ, Sch Med, Dept Pharmacol, Kwangju, South Korea
[5] Chonnam Natl Univ, Sch Med, Med Res Ctr Gene Regulat, Kwangju, South Korea
关键词
Hop; conduction; connexin40; Nkx2-5; mouse mutants;
D O I
10.1161/01.RES.0000163108.47258.f3
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Hop is an unusual homeobox gene expressed in the embryonic and adult heart. Hop acts downstream of Nkx2-5 during development, and Nkx2-5 mutations are associated with cardiac conduction system (CCS) defects. Inactivation of Hop in the mouse is lethal in half of the expected null embryos. Here, we show that Hop is expressed strongly in the adult CCS. Hop(-/-) adult mice display conduction defects below the atrioventricular node (AVN) as determined by invasive electrophysiological testing. These defects are associated with decreased expression of connexin40. Our results suggest that Hop functions in the adult CCS and demonstrate conservation of molecular hierarchies between embryonic myocardium and the specialized conduction tissue of the mature heart.
引用
收藏
页码:898 / 903
页数:6
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