Apelin (APLN), angiotensin I converting enzyme 2 (ACE2) and angiotensin II receptor type 2 (ATGR2) cause vasodilatation and normally inhibit the development of pulmonary hypertension and in each case reductions of protein expression have been shown to contribute to the development of disease [14,91,92,93,94,95,96,97]. Here, ACE2 is linked to pulmonary hypertension.