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Báo cáo y học: "Bone morphogenetic proteins, genetics and the pathophysiology of primary pulmonary hypertension"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học 'Respiratory Research cung cấp cho các bạn kiến thức về ngành y đề tài:Bone morphogenetic proteins, genetics and the pathophysiology of primary pulmonary hypertension" | Available online http respiratory-research.eom content 2 4 193 Commentary Bone morphogenetic proteins genetics and the pathophysiology of primary pulmonary hypertension Mark De Caestecker and Barbara Meyrick Department of Medicine Vanderbilt University Medical Center Nashville Tennessee USA Department of Pathology Vanderbilt University Medical Center Nashville Tennessee USA Correspondence Barbara Meyrick PhD Professor of Pathology and Medicine Center for Lung Research Vanderbilt University Medical Center Nashville TN 37232-2650 USA. Tel 1 615 322 3412 fax 1 615 343 7448 e-mail barbara.meyrick@mcmcail.vanderbilt.edu o 3 I Received 2 April 2001 Revisions requested 20 April 2001 Revisions received 17 May 2001 Accepted 17 May 2001 Published 11 June 2001 Respir Res 2001 2 193-197 2001 BioMed Central Ltd Print ISSN 1465-9921 Online ISSN 1465-993X Abstract 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 secreted growth factors known as the bone morphogenetic proteins BMPs these findings suggest that BMPs play an important role in the maintenance of normal pulmonary vascular physiology. In the present commentary we discuss the implications of these findings in the context of BMP receptor biology and relate these data to the genetics and pulmonary pathophysiology of patients with PPH. Keywords bone morphogenetic protein type II receptor BMPR-II gene mutations morphology pulmonary arteries transforming growth factor TGF -p superfamily Introduction The diagnosis of PPH is made in the presence of an increase in pulmonary vascular resistance associated with right ventricular failure in the absence of any other disease process. The etiology and .