BMPR2 and pulmonary arterial hypertension: The F14, KDF and R899X mutants of BMPR2 correspond to hereditary PAH-disease associated autosomally-dominant mutants which are known to mislocalize in vascular cells and to inhibit BMP-responsive signaling in 293T cells [1,2,3,31](details of the mutations and the subcellular localization are in Materials and Methods, and in ref. [31]).