Pulmonary Artery Hypertension: Caveolin-1 and eNOS Interrelationship: A New PerspectiveMathew, Rajamma MD; Huang, Jing MD; Gewitz, Michael H. MDCardiology in Review: May-June 2007 - Volume 15 - Issue 3 - p 143-149 doi: 10.1097/01.crd.0000249381.49138.b9 Review Article Buy Abstract Author InformationAuthors Article MetricsMetrics 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. From the Section of Pediatric Cardiology, Maria Fareri Children's Hospital at Westchester Medical Center, New York Medical College, Valhalla, New York. Correspondence: Rajamma Mathew, MD, Section of Pediatric Cardiology, New York Medical College, Munger Pavilion, Valhalla, NY 10595. E-mail: rajamma_mathew@NYMC.edu. © 2007 Lippincott Williams & Wilkins, Inc.