The normal adult pulmonary circulation is a low-pressure, high-capacity circuit. Pulmonary vascular resistance is regulated by alveolar oxygen tension, potassium channels and a variety of locally produced and circulating vasoactive factors. Perturbations of these systems may contribute to the pathogenesis of pulmonary hypertension. Recently, mutations in BMPR2 and ALK-1, genes that encode members of the transforming growth factor-β (TGF-β) receptor superfamily, have been found in patients with primary pulmonary hypertension. These observations provide a novel insight into the pathogenesis of primary pulmonary hypertension, and emphasize the importance of the integrity of the TGF-β receptor family in the maintenance of normal pulmonary vascular structure and function. This review discusses the latest developments in the field of pulmonary vascular biology and the prospects for improving the treatment of pulmonary hypertension.
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Review Article|
February 11 2002
Recent insights into the pathogenesis and therapeutics of pulmonary hypertension
Julian W. STRANGE;
Julian W. STRANGE
1Section on Clinical Pharmacology, Imperial College, Hammersmith Hospital, Du Cane Road, London W12 ONN, U.K.
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John WHARTON;
John WHARTON
1Section on Clinical Pharmacology, Imperial College, Hammersmith Hospital, Du Cane Road, London W12 ONN, U.K.
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Peter G. PHILLIPS;
Peter G. PHILLIPS
1Section on Clinical Pharmacology, Imperial College, Hammersmith Hospital, Du Cane Road, London W12 ONN, U.K.
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Martin R. WILKINS
1Section on Clinical Pharmacology, Imperial College, Hammersmith Hospital, Du Cane Road, London W12 ONN, U.K.
Correspondence: Professor M. R. Wilkins (e-mail m.wilkins@ic.ac.uk).
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Publisher: Portland Press Ltd
Online ISSN: 1470-8736
Print ISSN: 0143-5221
The Biochemical Society and the Medical Research Society © 2002
2002
Clin Sci (Lond) (2002) 102 (3): 253–268.
Citation
Julian W. STRANGE, John WHARTON, Peter G. PHILLIPS, Martin R. WILKINS; Recent insights into the pathogenesis and therapeutics of pulmonary hypertension. Clin Sci (Lond) 1 March 2002; 102 (3): 253–268. doi: https://doi.org/10.1042/cs1020253
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