Targeting bone morphogenic protein receptor 2 (BMPR2) signalling to treat pulmonary arterial hypertension
نویسندگان
چکیده
منابع مشابه
Targeting Vascular Remodeling to Treat Pulmonary Arterial Hypertension.
Pulmonary arterial hypertension (PAH) describes a group of conditions with a common hemodynamic phenotype of increased pulmonary artery pressure, driven by progressive remodeling of small pulmonary arteries, leading to right heart failure and death. Vascular remodeling is the key pathological feature of PAH, but treatments targeting this process are lacking. In this review, we summarize importa...
متن کاملRelationship of BMPR2 mutations to vasoreactivity in pulmonary arterial hypertension.
BACKGROUND Vasoreactivity tests are fundamental in evaluating pulmonary arterial hypertension (PAH). Mutations of the transforming growth factor-beta type II receptor gene, BMPR2, predispose to the development of pulmonary hypertension and may alter the response to vasodilators. Previous investigations have not examined the relationship of BMPR2 mutations to vasoreactivity. METHODS AND RESULT...
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Methods O f a total of 21 patients with IPAH, 13 patients received the PADN procedure, and the other 8 patients who refused the PADN procedure were assigned to the control group. PADN was performed at the bifurcation of the main PA, and at the ostial right and left PA. Serial echocardiography, right heart catheterization, and a 6-min walk test (6MWT) were performed. The primary endpoints were t...
متن کاملOverexpression of human bone morphogenetic protein receptor 2 does not ameliorate monocrotaline pulmonary arterial hypertension.
Pulmonary arterial hypertension (PAH) is associated with mutations of bone morphogenetic protein receptor 2 (BMPR2), and BMPR2 expression decreases with the development of experimental PAH. Decreased BMPR2 expression and impaired intracellular BMP signaling in pulmonary artery (PA) smooth muscle cells (PASMC) suppresses apoptosis and promotes proliferation, thereby contributing to the pathogene...
متن کاملBone Morphogenetic Protein Receptor Type 2 Mutation in Pulmonary Arterial Hypertension
Pulmonary arterial hypertension (PAH) is a rapidly progressive and lethal disease, characterized by an increase in resistance of the pulmonary arterioles, causing an increased right ventricular (RV) afterload. The RV adapts to this increased load via several compensatory mechanisms, but these are not sufficient to prevent progression to RV dysfunction and failure, which is the predominant cause...
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ژورنال
عنوان ژورنال: European Respiratory Journal
سال: 2017
ISSN: 0903-1936,1399-3003
DOI: 10.1183/13993003.01370-2017