Genetic Ablation of the Bmpr2 Gene in Pulmonary Endothelium Is Sufficient to Predispose to Pulmonary Arterial Hypertension | Zendy

Kwonho Hong | Zendy; Young Jae Lee | Zendy; Eunji Lee | Zendy; Sung Ok Park | Zendy; Chul Ju Han | Zendy; Hideyuki Beppu | Zendy; En Li | Zendy; Mohan K. Raizada | Zendy; Kenneth D. Bloch | Zendy; S. Paul Oh | Zendy

Open Access

Genetic Ablation of the Bmpr2 Gene in Pulmonary Endothelium Is Sufficient to Predispose to Pulmonary Arterial Hypertension

Author(s) -

Kwonho Hong,

Young Jae Lee,

Eunji Lee,

Sung Ok Park,

Chul Ju Han,

Hideyuki Beppu,

En Li,

Mohan K. Raizada,

Kenneth D. Bloch,

S. Paul Oh

Publication year - 2008

Publication title -

circulation

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 7.795

H-Index - 607

eISSN - 1524-4539

pISSN - 0009-7322

DOI - 10.1161/circulationaha.107.736801

Subject(s) - bmpr2 , medicine , pathogenesis , pulmonary hypertension , pulmonary artery , right ventricular hypertrophy , cardiology , endothelium , lung , endothelial dysfunction , blood pressure , pathology , endocrinology , gene , biology , genetics , bone morphogenetic protein

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 pathogenesis, no direct in vivo evidence has been clearly presented. The present study was designed to investigate whether endothelial Bmpr2 deletion can predispose to PAH.

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