Research ArticlePhysiology

PERK inhibition attenuates vascular remodeling in pulmonary arterial hypertension caused by BMPR2 mutation

See allHide authors and affiliations

Science Signaling  26 Jan 2021:
Vol. 14, Issue 667, eabb3616
DOI: 10.1126/scisignal.abb3616

You are currently viewing the editor's summary.

View Full Text

Log in to view the full text

Log in through your institution

Log in through your institution

Not a PERK for pulmonary arterial hypertension

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), and Shimizu et al. focused on the role of the UPR kinase PERK in PAH pathology. Genetic ablation or pharmacological inhibition of PERK limited vascular remodeling in two different mouse models of PAH and prevented mice from developing right ventricular heart failure. These effects involved suppressing the activation of PDGFRβ-STAT1 signaling and glycolysis in pulmonary artery smooth muscle cells. Thus, targeting PERK may help limit the mortality associated with PAH.

View Full Text

Stay Connected to Science Signaling