title: Pulmonary artery hypertension: caveolin-1 and eNOS interrelationship: a new perspective.
abstract: Pulmonary artery hypertension (PAH) is a sequela of a number of disparate diseases, often with a fatal consequence. Endothelial dysfunction is considered to be an early event during the development of PAH. Impaired availability of bioactive nitric oxide (NO) is a key underlying feature in most forms of clinical and experimental PAH. NO, generated by catalytic activity of endothelial NO synthase (eNOS) on l-arginine, modulates vascular function and structure. For optimal activation, eNOS is targeted to caveolae, the flask-shaped invaginations found on the surface of plasmalemmal membrane of a variety of cells, including endothelial cells. Caveolin-1, the major coat protein of caveolae, regulates eNOS activity. Evidence is accumulating to suggest that caveolin-1 may play a significant role in the pathogenesis of PAH. This review is intended to summarize recent findings indicating a role for caveolin-1 and caveolin-1/eNOS interrelationship in PAH.
authors: Section of Pediatric Cardiology, Maria Fareri Children's Hospital at Westchester Medical Center, New York Medical College, Valhalla, New York, USA. rajamma_mathew@NYMC.edu
authors: Rajamma
authors: R
authors: Mathew
authors: Jing
authors: J
authors: Huang
authors: Michael H
authors: MH
authors: Gewitz
mesh_terms: D000818:Animals; D015415:Biomarkers / Q000032:analysis; D051242:Caveolin 1 / Q000378:metabolism*; D002462:Cell Membrane / Q000187:drug effects / Q000502:physiology; D049109:Cell Proliferation / Q000187:drug effects; D018450:Disease Progression; D004730:Endothelium, Vascular / Q000187:drug effects / Q000201:enzymology / Q000378:metabolism; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000401:mortality / Q000503:physiopathology; D008297:Male; D018919:Neovascularization, Physiologic / Q000187:drug effects; D009569:Nitric Oxide / Q000008:administration & dosage*; D019001:Nitric Oxide Synthase / Q000378:metabolism*; D018570:Risk Assessment; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D016019:Survival Analysis; D014655:Vascular Resistance
chemical_list: D015415:Biomarkers; D051242:Caveolin 1; D009569:Nitric Oxide; D019001:Nitric Oxide Synthase