Bone Morphogenetic Protein Receptor Type 2 Mutation in Pulmonary Arterial Hypertension
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چکیده
منابع مشابه
Bone 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...
متن کاملBone Morphogenetic Protein Receptor Type 2 Mutation in Pulmonary Arterial Hypertension: A View on the Right Ventricle.
BACKGROUND The effect of a mutation in the bone morphogenetic protein receptor 2 (BMPR2) gene on right ventricular (RV) pressure overload in patients with pulmonary arterial hypertension is unknown. Therefore, we investigated RV function in patients who have pulmonary arterial hypertension with and without the BMPR2 mutation by combining in vivo measurements with molecular and histological anal...
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Unraveling a Riddle Inside an Enigma? Bone Morphogenetic Protein Receptor-2 and Pulmonary Arterial Hypertension: Print ISSN: 0009-7330. Online ISSN: 1524-4571 Copyright © 2005 American Heart Association, Inc. All rights reserved. is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Circulation Research doi: 10.1161/01.RES.0000168922.54339.47 2005;96:1033-1035...
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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...
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Idiopathic pulmonary arterial hypertension (PAH) is characterized by proliferation of pulmonary vascular endothelial and smooth muscle cells causing increased vascular resistance and right heart failure. Mutations in the bone morphogenetic protein receptor type 2 (BMPR2) are believed to cause the familial form of the disease. Reduced expression of BMPR2 is also noted in secondary PAH. Recent ad...
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ژورنال
عنوان ژورنال: Circulation
سال: 2016
ISSN: 0009-7322,1524-4539
DOI: 10.1161/circulationaha.115.020696