Substance P Promotes Pulmonary Hypertension by Inducing Fibulin-2 Methylation to Regulate β-Catenin.

ABSTRACT: Pulmonary arterial hypertension (PAH) is a fatal disease with limited available treatments and is characterized by pulmonary vascular remodeling. Substance P (SP) may be involved in vascular remodeling in patients with PAH. However, the underlying mechanism is currently unknown. In this study, we found that plasma SP levels were elevated and correlated with pulmonary hemodynamic parameters in PAH patients. SP receptor inhibitors significantly suppressed pulmonary vascular remodeling and improved pulmonary circulation hemodynamics in PAH rats. Multiple omics analyses suggested that downregulation of Fibulin-2 (Fbln2) may play a role in promoting pulmonary vascular remodeling by SP. In addition, the downregulation of Fbln2 expression by SP was further verified by Western blot analysis and im

SUBMITTER: Yi W 

PROVIDER: S-EPMC12619955 | biostudies-literature | 2025 Oct

REPOSITORIES: biostudies-literature