abt-100 and Rib-Fractures

abt-100 has been researched along with Rib-Fractures* in 1 studies

Other Studies

1 other study(ies) available for abt-100 and Rib-Fractures

Article	Year
A protein farnesyltransferase inhibitor ameliorates disease in a mouse model of progeria. Science (New York, N.Y.), 2006, Mar-17, Volume: 311, Issue:5767 Progerias are rare genetic diseases characterized by premature aging. Several progeroid disorders are caused by mutations that lead to the accumulation of a lipid-modified (farnesylated) form of prelamin A, a protein that contributes to the structural scaffolding for the cell nucleus. In progeria, the accumulation of farnesyl-prelamin A disrupts this scaffolding, leading to misshapen nuclei. Previous studies have shown that farnesyltransferase inhibitors (FTIs) reverse this cellular abnormality. We tested the efficacy of an FTI (ABT-100) in Zmpste24-deficient mice, a mouse model of progeria. The FTI-treated mice exhibited improved body weight, grip strength, bone integrity, and percent survival at 20 weeks of age. These results suggest that FTIs may have beneficial effects in humans with progeria. Topics: Animals; Body Weight; Disease Models, Animal; Enzyme Inhibitors; Farnesyltranstransferase; Female; Hand Strength; Imidazoles; Lamin Type A; Male; Membrane Proteins; Metalloendopeptidases; Mice; Nuclear Proteins; Progeria; Protein Precursors; Protein Prenylation; Rib Fractures; Survival Rate	2006

Article

Year

A protein farnesyltransferase inhibitor ameliorates disease in a mouse model of progeria.

Science (New York, N.Y.), 2006, Mar-17, Volume: 311, Issue:5767

Progerias are rare genetic diseases characterized by premature aging. Several progeroid disorders are caused by mutations that lead to the accumulation of a lipid-modified (farnesylated) form of prelamin A, a protein that contributes to the structural scaffolding for the cell nucleus. In progeria, the accumulation of farnesyl-prelamin A disrupts this scaffolding, leading to misshapen nuclei. Previous studies have shown that farnesyltransferase inhibitors (FTIs) reverse this cellular abnormality. We tested the efficacy of an FTI (ABT-100) in Zmpste24-deficient mice, a mouse model of progeria. The FTI-treated mice exhibited improved body weight, grip strength, bone integrity, and percent survival at 20 weeks of age. These results suggest that FTIs may have beneficial effects in humans with progeria.

Topics: Animals; Body Weight; Disease Models, Animal; Enzyme Inhibitors; Farnesyltranstransferase; Female; Hand Strength; Imidazoles; Lamin Type A; Male; Membrane Proteins; Metalloendopeptidases; Mice; Nuclear Proteins; Progeria; Protein Precursors; Protein Prenylation; Rib Fractures; Survival Rate

2006