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Genetic ablation of the Bmpr2 gene in pulmonary endothelium is sufficient to predispose to pulmonary arterial hypertension

BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare but fatal lung disease of diverse etiologies. PAH is now further subclassified as idiopathic (IPAH), familial (FPAH) and associated (APAH) varieties. Heterozygous mutations in BMPR2 can be detected in 50-70% of patients with FPAH and 10-40%...

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Detalhes bibliográficos
Main Authors: Hong, Kwon-Ho, Lee, Young Jae, Lee, Eunji, Park, Sung Ok, Han, Chul, Beppu, Hideyuki, Li, En, Raizada, Mohan, Bloch, Kenneth D., Oh, S. Paul
Formato: Artigo
Idioma:Inglês
Publicado em: 2008
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3920834/
https://ncbi.nlm.nih.gov/pubmed/18663089
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1161/CIRCULATIONAHA.107.736801
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