The two-faced progeria gene and its implications in aging and metabolism

EMBO Rep. 2014 May;15(5):470-1. doi: 10.1002/embr.201438776. Epub 2014 Apr 10.

Abstract

Premature aging syndromes have gained much attention, not only because of their devastating symptoms but also because they might hold a key to some of the mechanisms underlying aging. The Hutchinson-Gilford progeria syndrome (HGPS) is caused by a mutation in the LMNA gene, which normally produces lamins A and C through alternative splicing. Due to this mutation, HGPS patients express an incompletely processed form of lamin A called progerin. In this issue of EMBO Reports, the Tazi group demonstrates how mice expressing different LMNA isoforms present opposite phenotypes in longevity, fat storage and mitochondrial function.

Publication types

  • Comment

MeSH terms

  • Adipose Tissue / physiology*
  • Animals
  • Energy Metabolism / genetics*
  • Lamin Type A / genetics*
  • Lamin Type A / metabolism*

Substances

  • Lamin Type A
  • Lmna protein, mouse
  • lamin C