Pharmacologic activation of Δ133p53α reduces cellular senescence in progeria patients-derived cells.

ABSTRACT:

Background

Patients with Hutchinson-Gilford progeria syndrome (HGPS) show accelerated aging phenotypes and have shortened lifespan, with implications in physiological aging processes as well. While therapeutic approaches targeting the disease-causing abnormal protein, progerin, have been developed, further efforts to explore mechanistically distinct and complementary strategies are still critical to better treatment regimens. We previously showed that lentiviral vector-driven expression of Δ133p53α, a natural inhibitory isoform of p53, rescued HGPS patients-derived fibroblasts from early entry into cellular senescence, which is a downstream event of progerin-induced DNA damage. We also performed a quantitative high-throughput screen (qHTS) of approved drug and investigational agent

SUBMITTER: Joruiz SM 

PROVIDER: S-EPMC12467803 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature