Recent insights into the pathogenesis and therapeutics of pulmonary hypertension

被引：25
|
作者：
Strange, JW [1 ]
Wharton, J [1 ]
Phillips, PG [1 ]
Wilkins, MR [1 ]
机构：
[1] Hammersmith Hosp, Imperial Coll, Clin Pharmacol Sect, London W12 0NN, England
来源：
CLINICAL SCIENCE | 2002年 / 102卷 / 03期
关键词：
BMP type II receptor; genetic predisposition; hypoxia; pulmonary hypertension; therapeutics; transforming growth; factor beta receptor;
D O I：
10.1042/CS20010212
中图分类号：
R-3 [医学研究方法]; R3 [基础医学];
学科分类号：
1001 ;
摘要：
The normal adult pulmonary circulation is a low-pressure, high-capacity circuit. Pulmonary vascular resistance is regulated by alveolar oxygen tension, potassium channels and a variety of locally produced and circulating vasoactive factors. Perturbations of these systems may contribute to the pathogenesis of pulmonary hypertension. Recently, mutations in BMPR2 and ALK-1, genes that encode members of the transforming growth factor-beta (TGF-beta) receptor superfamily, have been found in patients with primary pulmonary hypertension. These observations provide a novel insight into the pathogenesis of primary pulmonary hypertension, and emphasize the importance of the integrity of the TGF-beta receptor family in the maintenance of normal pulmonary vascular structure and function. This review discusses the latest developments in the field of pulmonary vascular biology and the prospects for improving the treatment of pulmonary hypertension.
引用
收藏
页码：253 / 268
页数：16
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