BMPR2 and pulmonary arterial hypertension: For example, Pulmonary Arterial Hypertension (PAH) results in abnormal vascular remodeling and hypertension and is linked to BMPR2 mutations in patients, and mice lacking one copy of Bmpr2 had mild pulmonary hypertension and impaired pulmonary vascular remodeling under hypoxic conditions, a phenotype reminiscent of human PAH [171].