Using a mouse model of premature, rapid aging (Hutchinson-Gilford progeria syndrome), Ibrahim and colleagues show that methylated prelamin A, an intermediate form in the protein’s maturation pathway, is associated with premature aging symptoms. In this disorder, patients fail to thrive, and death occurs at an average age of 13 years, usually from myocardial infarction or stroke.  LMNA, the gene harboring the Hutchinson-Gilford progeria mutations, encodes prelamin A which matures to lamin A, a structural component of the nuclear envelope. The methylated form of prelamin A blocks a signaling pathway that promotes cell proliferation, survival, and tissue growth. The report suggests that reducing this methylation pharmacologically could be an effective treatment for progeroid premature aging disorders.

Ibrahim MX, Sayin VI, Akula MK, Liu M, Fong LG, Young SG, Bergo MO: Targeting Isoprenylcysteine Methylation Ameliorates Disease in a Mouse Model of Progeria. Science 340 (6138): 1330-1333 (2013).

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