Article
Genetic ablation of the BMPR2 gene in pulmonary endothelium is sufficient to predispose to pulmonary arterial hypertension.
Circulation - 12 Aug 2008
Hong Kwon-Ho, Lee Young Jae, Lee Eunji, Park Sung Ok, Han Chul, Beppu Hideyuki, Li En, Raizada Mohan K, Bloch Kenneth D, Oh S Paul
Abstract excerpt
BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare but fatal lung disease of diverse origins. PAH is now further subclassified as idiopathic PAH, familial PAH, and associated PAH varieties. Heterozygous mutations in BMPR2 can be detected in 50% to 70% of patients with familial PAH and 10% to 40% of patients with idiopathic PAH. Although endothelial cells have been suspected as the cellular origin of PAH...
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