Expression of Type II Bone Morphogenetic Protein Receptor Primary Pulmonary Hypertension Is Associated With Reduced Pulmonary Vascular
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چکیده
Carl Atkinson, Susan Stewart, Paul D. Upton, Rajiv Machado, Jennifer R. Thomson, Richard C. Expression of Type II Bone Morphogenetic Protein Receptor Primary Pulmonary Hypertension Is Associated With Reduced Pulmonary Vascular Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 2002 American Heart Association, Inc. All rights reserved. is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Circulation doi: 10.1161/01.CIR.0000012754.72951.3D 2002;105:1672-1678; originally published online March 25, 2002; Circulation. http://circ.ahajournals.org/content/105/14/1672 World Wide Web at: The online version of this article, along with updated information and services, is located on the
منابع مشابه
Primary pulmonary hypertension is associated with reduced pulmonary vascular expression of type II bone morphogenetic protein receptor.
BACKGROUND Mutations in the type II receptor for bone morphogenetic protein (BMPR-II), a receptor member of the transforming growth factor-beta (TGF-beta) superfamily, underlie many familial and sporadic cases of primary pulmonary hypertension (PPH). METHODS AND RESULTS Because the sites of expression of BMPR-II in the normal and hypertensive lung are unknown, we studied the cellular localiza...
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Pulmonary arterial hypertension (PAH) remains a fatal disease despite modern pharmacotherapy. Mutations in the gene for bone morphogenetic protein receptor type II (BMPR2) lead to reduced BMPR2 expression, which is causally linked to PAH. BMPR2 is predominantly expressed on pulmonary endothelium and has complex interactions with transforming growth factor (TGF)-β signalling mechanisms. Our obje...
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Several recent papers have shown that both familial primary pulmonary hypertension (FPPH) and sporadic primary pulmonary hypertension (PPH) may have a common etiology that is associated with the inheritance and/or spontaneous development of germline mutations in the bone morphogenetic protein receptor (BMPR) type II gene. Because BMPR-II is a ubiquitously expressed receptor for a family of secr...
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