To test whether our findings in BMPR2-deficient cells regarding the deposition of ectopic FBN1 have the same relevance in human tissues, we next analyzed BMPR2 expression in lung samples from patients with documented forms of IPAH or familiar PAH (heritable PAH [HPAH]) (Fig 7A). This evidence concerns the gene BMPR2 and idiopathic pulmonary arterial hypertension.