Treatment with a farnesyltransferase inhibitor improves survival in mice with a Hutchinson–Gilford progeria syndrome mutation

Abstract

Hutchinson–Gilford progeria syndrome (HGPS) is a progeroid syndrome characterized by multiple aging-like disease phenotypes. We recently reported that a protein farnesyltransferase inhibitor (FTI) improved several disease phenotypes in mice with a HGPS mutation ( Lmna HG/+). Here, we investigated the impact of an FTI on the survival of Lmna HG/+ mice. The FTI significantly improved the survival of both male and female Lmna HG/+ mice. Treatment with the FTI also improved body weight curves and reduced the number of spontaneous rib fractures. This study provides further evidence for a beneficial effect of an FTI in HGPS.