Fig 1.

(A) Right ventricular systolic pressures are significantly elevated in BMPR2 mutant mice with six weeks of transgene activation using doxycycline at 1g/kg in western diet; this elevation was prevented through administration of SB2014741 in pumps for the final four weeks. Circles represent individual mice; columns are averages of log₂-transformed values; error bars are SEM. SB204741 did not affect control mice; both vehicle and treated mice are included in the control column as left and right groups of circles, respectively. (B) Cardiac Index does not change between groups, measured as cardiac output in ml/minute as determined by echocardiography divided by body surface area in square meters. (C) Immunoflourescence staining for CD45 in a 10x field of distal alveoli in agarose-inflated lungs. An increase in CD45+ cells is immediately apparent with BMPR2 mutation and with SB204741 treatment in controls. (D) BMPR2 mutant mice have ~2x the inflammatory cells per field at baseline, but SB204741 treatment has divergent effects on inflammatory cells in control and BMPR2 mutant mice, causing significant increases and decreases respectively (* = p<0.05, § = p<0.01). (E) BMPR2 mutant mice have roughly twice the numbers of partially and fully muscularized vessels per field for small and medium sized vessels; this is substantially normalized by SB204741 (* = p<0.01).