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.
Exogenous H<sub>2</sub>S suppressed ER stress indexes <i>in vivo</i> and <i>in vitro</i>, decreased activating transcription factor 6 activation, and inhibited the hypoxia-induced decrease in mitochondrial calcium and mitochondrial function.<b>Conclusion:</b> H<sub>2</sub>S effectively inhibits hypoxia-induced increase in cell proliferation, migration, and oxidative stress in PASMCs, and NOX-4 might be the underlying mechanism of PAH.
Collectively, reports indicate that Nox4 contributes to altered fibroblast behavior, ROS production leading to hypertensive vascular remodeling and the development of PAH.
Our results showed that the serum Gal-3 and NADPH oxidase 4 (Nox4) levels were significantly elevated and correlated in 26 human PAH patients when compared with 14 age- and sex-matched healthy controls.