A Protein Farnesyltransferase Inhibitor Ameliorates Disease in a Mouse Model of Progeria

@article{Fong2006APF,
  title={A Protein Farnesyltransferase Inhibitor Ameliorates Disease in a Mouse Model of Progeria},
  author={L. Fong and D. Frost and M. Meta and X. Qiao and S. Yang and Catherine Coffinier and S. Young},
  journal={Science},
  year={2006},
  volume={311},
  pages={1621 - 1623}
}
  • L. Fong, D. Frost, +4 authors S. Young
  • Published 2006
  • Medicine, Biology
  • Science
  • 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… CONTINUE READING
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