Mutations in BMPR2 structure leads to a decrease in BMPR2 expression and are present in approximately 70% of patients with familial PAH and 20% of patients with sporadic idiopathic PAH, being implied with the initiation and progression of PAH, involved in the regulation of vascular remodeling and inflammation in the lung (50–52). Here, BMPR2 is linked to pulmonary arterial hypertension.