TNFAIP3 and pulmonary arterial hypertension: A central role in the immune modulation of PH is attributed to the key regulatory ubiquitin-binding protein A20 (encoded by TNFAIP3), as polymorphisms in the TNFAIP3 locus are strongly associated with the development of PAH (Dieude et al., 2010; Ma and Malynn, 2012).