Document Detail


Molecular pathogenesis of pulmonary arterial hypertension.
MedLine Citation:
PMID:  23202738     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Recent clinical and experimental studies are redefining the cellular and molecular bases of pulmonary arterial hypertension (PAH). The genetic abnormalities first identified in association with the idiopathic form of PAH--together with a vast increase in our understanding of cell signaling, cell transformation, and cell-cell interactions; gene expression; microRNA processing; and mitochondrial and ion channel function--have helped explain the abnormal response of vascular cells to injury. Experimental and clinical studies now converge on the intersection and interactions between a genetic predisposition involving the BMPR2 signaling pathway and an impaired metabolic and chronic inflammatory state in the vessel wall. These deranged processes culminate in an exuberant proliferative response that occludes the pulmonary arterial (PA) lumen and obliterates the most distal intraacinar vessels. Here, we describe emerging therapies based on preclinical studies that address these converging pathways.
Authors:
Marlene Rabinovitch
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Publication Detail:
Type:  Journal Article; Review     Date:  2012-12-03
Journal Detail:
Title:  The Journal of clinical investigation     Volume:  122     ISSN:  1558-8238     ISO Abbreviation:  J. Clin. Invest.     Publication Date:  2012 Dec 
Date Detail:
Created Date:  2012-12-03     Completed Date:  2013-02-04     Revised Date:  2014-03-28    
Medline Journal Info:
Nlm Unique ID:  7802877     Medline TA:  J Clin Invest     Country:  United States    
Other Details:
Languages:  eng     Pagination:  4306-13     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Animals
Bone Morphogenetic Protein Receptors, Type II / genetics,  physiology
Estrogens / metabolism
Glycolysis
Humans
Hypertension, Pulmonary / genetics,  metabolism*,  physiopathology*
Mutation
Pancreatic Elastase / metabolism
Receptors, Serotonin / metabolism
Serotonin Plasma Membrane Transport Proteins / metabolism
Signal Transduction
Stem Cells / metabolism
Grant Support
ID/Acronym/Agency:
P01 HL108797/HL/NHLBI NIH HHS; R01 HL074186/HL/NHLBI NIH HHS
Chemical
Reg. No./Substance:
0/Estrogens; 0/Receptors, Serotonin; 0/Serotonin Plasma Membrane Transport Proteins; EC 2.7.11.30/BMPR2 protein, human; EC 2.7.11.30/Bone Morphogenetic Protein Receptors, Type II; EC 3.4.21.36/Pancreatic Elastase
Comments/Corrections

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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