Discovery of a murine model of clinical PAH: Mission impossible?
Discovery of a murine model of clinical PAH: Mission impossible?
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DOI：
10.1016/j.tcm.2016.12.003
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发表时间：
2017-05-01
影响因子：
9.3
通讯作者：
Zhao, You-Yang
中科院分区：
文献类型：
--
作者：
Dai, Zhiyu;Zhao, You-Yang
肺动脉高压（Pulmonary arterial hypertension， PAH）是一种肺部血管疾病，其特征是肺血管阻力进行性增加，肺血管重构闭塞，导致右心衰竭和过早死亡。在这篇简短的综述中，我们记录了鉴定基因修饰小鼠PH模型的最新进展，重点是最近发现的Tie2 cre介导的脯氨酰羟化酶2缺失小鼠模型，该模型表现出进行性血管阻塞重构、严重PAH和右心衰，从而概括了临床PAH的许多特征。我们还将讨论从小鼠PH模型的实验研究中产生的最新发现的转化潜力。
Pulmonary arterial hypertension (PAH) is a lung vascular disease characterized with a progressive increase of pulmonary vascular resistance and obliterative pulmonary vascular remodeling resulting in right heart failure and premature death. In this brief review, we document the recent advances in identifying genetically modified murine models of PH, with a focus on the recent discovery of the mouse model of Tie2 Cre-mediated deletion of prolyl hydroxylase 2, which exhibits progressive obliterative vascular remodeling, severe PAH, and right heart failure, thus recapitulating many of the features of clinical PAH. We will also discuss the translational potential of recent findings arising from experimental studies of murine PH models.