PerspectiveCell Biology

Rapid Aging Rescue?

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Science  14 Jun 2013:
Vol. 340, Issue 6138, pp. 1299-1300
DOI: 10.1126/science.1240843

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Summary

Hutchinson-Gilford progeria syndrome (HGPS) is a rare dominant genetic disorder that mimics premature, rapid aging. Patients fail to thrive, and death occurs at an average age of 13 years, usually from myocardial infarction or stroke. Spontaneous HGPS-generating mutations occur at the rate of about 1 in 4 to 8 million births. LMNA, the gene harboring the HGPS mutations, encodes prelamin A (1). This precursor matures to lamin A, a structural component of the nuclear envelope. On page 1330 of this issue, Ibrahim et al. (2) show that methylated prelamin A, an intermediate form in the protein's maturation pathway, is associated with progeria symptoms in a mouse model of the disease. The methylated form blocks a signaling pathway that promotes cell proliferation, survival, and tissue growth. Reducing this methylation pharmacologically could be an effective therapeutic approach for treating progeroid disorders.