Document Detail

Recent insights into the pathogenesis and therapeutics of pulmonary hypertension.
MedLine Citation:
PMID:  11869166     Owner:  NLM     Status:  MEDLINE    
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-beta (TGF-beta) 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-beta 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.
Julian W Strange; John Wharton; Peter G Phillips; Martin R Wilkins
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Clinical science (London, England : 1979)     Volume:  102     ISSN:  0143-5221     ISO Abbreviation:  Clin. Sci.     Publication Date:  2002 Mar 
Date Detail:
Created Date:  2002-02-28     Completed Date:  2002-03-25     Revised Date:  2012-06-25    
Medline Journal Info:
Nlm Unique ID:  7905731     Medline TA:  Clin Sci (Lond)     Country:  England    
Other Details:
Languages:  eng     Pagination:  253-68     Citation Subset:  IM    
Section on Clinical Pharmacology, Imperial College, Hammersmith Hospital, Du Cane Road, London W12 ONN, UK.
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MeSH Terms
Anoxia / complications
Bone Morphogenetic Protein Receptors, Type II
Gene Therapy
Hypertension, Pulmonary / etiology*,  therapy*
Potassium Channels / metabolism
Protein-Serine-Threonine Kinases / genetics
Pulmonary Artery / pathology
Pulmonary Disease, Chronic Obstructive / complications
Vascular Resistance
Vasodilator Agents / therapeutic use
Reg. No./Substance:
0/Potassium Channels; 0/Vasodilator Agents; EC Kinases; EC protein, human; EC Morphogenetic Protein Receptors, Type II
Comment In:
Clin Sci (Lond). 2002 Oct;103(4):345; author reply 346   [PMID:  12241531 ]

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