Document Detail

Molecular pathogenesis of pulmonary arterial hypertension.
MedLine Citation:
PMID:  23202738     Owner:  NLM     Status:  MEDLINE    
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.
Marlene Rabinovitch
Related Documents :
23783368 - Emerging patterns of the human superior thyroid artery and review of its clinical anatomy.
23581588 - Charles stent and the mystery behind the word "stent"
24436588 - Subclavian aneurysm presenting with massive hemoptysis: a case report and review of the...
105808 - A rapidly polymerizing polyurethane for transcatheter embolization.
16755348 - Congenital aorto-azygous fistula treated with coil embolization: case report and review...
11233248 - Minimally invasive coronary artery bypass grafting surgery in a child with kawasaki dis...
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    
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Bone Morphogenetic Protein Receptors, Type II / genetics,  physiology
Estrogens / metabolism
Hypertension, Pulmonary / genetics,  metabolism*,  physiopathology*
Pancreatic Elastase / metabolism
Receptors, Serotonin / metabolism
Serotonin Plasma Membrane Transport Proteins / metabolism
Signal Transduction
Stem Cells / metabolism
Grant Support
Reg. No./Substance:
0/Estrogens; 0/Receptors, Serotonin; 0/Serotonin Plasma Membrane Transport Proteins; EC protein, human; EC Morphogenetic Protein Receptors, Type II; EC Elastase

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

Previous Document:  CDX2-driven leukemogenesis involves KLF4 repression and deregulated PPAR? signaling.
Next Document:  Fragile X syndrome: causes, diagnosis, mechanisms, and therapeutics.