BMPR2 and pulmonary arterial hypertension: The expression of a BMPR2 mutant protein in human lung VECs contributes to the progress of PAH mainly by subsequent metabolic changes in VECs: (1) upregulated expression of glycolysis-related enzymes; (2) depressed carnitine metabolism and fatty acid oxidation;, and (3) decreased TCA cycle intermediates (Fessel et al., 2012).