To explore the functional consequences of BMPR2 mutations located at the different domains, we generated four mutants of BMPR2 identified in patients with PAH: Tyr67Cys in the extracellular ligand-binding domain, Thr268fs and Gln433X in the serine-threonine KD, and Ser863Asn in the C-terminal domain. Here, BMPR2 is linked to pulmonary arterial hypertension.