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

SH Yang, X Qiao, LG Fong, SG Young - Biochimica et Biophysica Acta …, 2008 - Elsevier
SH Yang, X Qiao, LG Fong, SG Young
Biochimica et Biophysica Acta (BBA)-Molecular and Cell Biology of Lipids, 2008Elsevier
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 (LmnaHG/+). Here, we investigated the impact of an FTI on the survival of
LmnaHG/+ mice. The FTI significantly improved the survival of both male and female
LmnaHG/+ mice. Treatment with the FTI also improved body weight curves and reduced the …
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 (LmnaHG/+). Here, we investigated the impact of an FTI on the survival of LmnaHG/+ mice. The FTI significantly improved the survival of both male and female LmnaHG/+ 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.
Elsevier