Article
Functional changes in pulmonary arterial endothelial cells associated with BMPR2 mutations.
PloS one - 1 Jan 2014
Wang Hu, Ji Ruirui, Meng Jie, Cui Qiqiong, Zou Wenxin, Li Lei, Wang Guoliang, Sun Li, Li Zhaohui, Huo Lei, Fan Yuxin, Penny Daniel J
Abstract excerpt
Pulmonary arterial hypertension (PAH) is a devastating disease characterized by abnormal remodeling of small, peripheral pulmonary arteries. Germline mutations in the bone morphogenetic protein receptor type 2 (BMPR2) gene are a major risk factor for developing PAH. At present, the correlation between the BMPR2 mutation and the patient's prognosis remains controversial despite several investigations. In this...
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