Bone Morphogenetic Protein Receptor-2 and Pulmonary Arterial Hypertension
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چکیده
منابع مشابه
Bone morphogenetic protein receptor-2 and pulmonary arterial hypertension: unraveling a riddle inside an enigma?
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...
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...
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 کاملFailure of bone morphogenetic protein receptor trafficking in pulmonary arterial hypertension: potential for rescue.
Heterozygous germline mutations in the gene encoding the bone morphogenetic protein type II receptor cause familial pulmonary arterial hypertension (PAH). We previously demonstrated that the substitution of cysteine residues in the ligand-binding domain of this receptor prevents receptor trafficking to the cell membrane. Here we demonstrate the potential for chemical chaperones to rescue cell-s...
متن کامل[Mutations in the gene encoding bone morphogenetic protein receptor 2 in patients with idiopathic pulmonary arterial hypertension].
OBJECTIVE Pulmonary arterial hypertension (PAH) is a rare disease that can have a familial component. It has been shown that more than 50% of cases of familial PAH are associated with mutations in the gene encoding bone morphogenetic protein receptor 2 (BMPR2), which acts as a receptor for members of the transforming growth factor beta superfamily. Some studies in patients with idiopathic PAH h...
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ژورنال
عنوان ژورنال: Circulation Research
سال: 2005
ISSN: 0009-7330,1524-4571
DOI: 10.1161/01.res.0000168922.54339.47