Figure 3.

Analysis of targeted gene expression profiles of skin mesenchymal cells from PAH BMPR2 and CAV1 mutation carriers relative to wild-type individuals (i.e., noncarriers of mutations) and unaffected mutation carriers. A, A qRT-PCR analysis was performed to validate the patterns of representative genes expressed by skin MCs; n = 3 patients for each group. The mean of combined patient samples per group and results for individual samples are presented. All amplification was normalized to a housekeeping gene, and the results are presented as mean fold change over control ± standard error. B, Representative immunofluorescent images of DKK1 and PDK4 localization in skin MCs and lung MPCs. Scale bar: 100 μm. DAPI: 4′,6-diamidino-2-phenylindole; HPAH: heritable PAH; IPAH: idiopathic PAH; MCs: mesenchymal cells; MPCs: mesenchymal progenitor cells; mut: mutant; PAH: pulmonary arterial hypertension; qRT-PCR: quantitative reverse transcription polymerase chain reaction.