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Publication : Treatment with a farnesyltransferase inhibitor improves survival in mice with a Hutchinson-Gilford progeria syndrome mutation.

First Author  Yang SH Year  2008
Journal  Biochim Biophys Acta Volume  1781
Issue  1-2 Pages  36-9
PubMed ID  18082640 Mgi Jnum  J:133416
Mgi Id  MGI:3778551 Doi  10.1016/j.bbalip.2007.11.003
Citation  Yang SH, et al. (2008) Treatment with a farnesyltransferase inhibitor improves survival in mice with a Hutchinson-Gilford progeria syndrome mutation. Biochim Biophys Acta 1781(1-2):36-9
abstractText  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.
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