Abstract

Physiology The vascular remodeling that occurs in pulmonary arterial hypertension (PAH) is eventually fatal because it leads to right ventricular heart failure. PAH induces the unfolded protein response (UPR) in vascular smooth muscle cells, which led Shimizu et al. to investigate the role of the UPR kinase PERK in disease pathology. Genetic ablation or pharmacological inhibition of PERK limited vascular remodeling in two different mouse models of PAH, preventing mice from developing right ventricular heart failure and improving survival. Sci. Signal. 14 , eabb3616 (2021).

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