肺动脉高压
基因亚型
缺氧(环境)
心力衰竭
发病机制
缺氧诱导因子
肺动脉
信号转导
医学
条件基因敲除
基因剔除小鼠
右心衰竭
内科学
生物信息学
生物
细胞生物学
化学
表型
受体
生物化学
基因
氧气
有机化学
作者
Soni Savai Pullamsetti,Argen Mamazhakypov,Norbert Weißmann,Werner Seeger
摘要
Pulmonary hypertension (PH) is characterized by pulmonary artery remodeling that can subsequently culminate in right heart failure and premature death. Emerging evidence suggests that hypoxia-inducible factor (HIF) signaling plays a fundamental and pivotal role in the pathogenesis of PH. This Review summarizes the regulation of HIF isoforms and their impact in various PH subtypes, as well as the elaborate conditional and cell-specific knockout mouse studies that brought the role of this pathway to light. We also discuss the current preclinical status of pan- and isoform-selective HIF inhibitors, and propose new research areas that may facilitate HIF isoform-specific inhibition as a novel therapeutic strategy for PH and right heart failure.
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