Pioglitazone inhibited the canonical TGFβ1-CTGF axis in human pulmonary artery SMC and smLRP1<sup>-/-</sup> main pulmonary artery (CTGF and NOX4) and reversed PAH in smLRP1<sup>-/-</sup> mice.
The present study suggests that PCPA protects against the pathogenesis of PAH by suppressing remodeling and inducing apoptosis, which are likely associated with CTGF and downstream ERK signaling pathway in rats.