Interestingly, a number of the X encoded genes listed in Table 1 that participate in PH development (including MAOA, NOX2, G6PD, ATP7A, XIAP, IKBKG, TIMP1, TMSB4X, FLNA, XIST) escape inactivation in other contexts although whether this occurs in the different forms of pulmonary hypertension has not been investigated to date [60,61,154,155,156,157]. Here, ATP7A is linked to pulmonary hypertension.