Aging and nuclear organization: lamins and progeria

Curr Opin Cell Biol. 2004 Jun;16(3):322-7. doi: 10.1016/


The discoveries of at least eight human diseases arising from mutations in LMNA, which encodes the nuclear A-type lamins, have revealed the nuclear envelope as an organelle associated with a variety of fundamental cellular processes. The most recently discovered diseases associated with LMNA mutations are the premature aging disorders Hutchinson-Gilford progeria syndrome (HGPS) and atypical Werner's syndrome. The phenotypes of both HGPS patients and a mouse model of progeria suggest diverse compromised tissue functions leading to defects reminiscent of aging. Aspects of the diseases associated with disrupted nuclear envelope/lamin functions may be explained by decreased cellular proliferation, loss of tissue repair capability and a decline in the ability to maintain a differentiated state.

Publication types

  • Review

MeSH terms

  • Animals
  • Cell Nucleus / metabolism*
  • Cellular Senescence / physiology
  • Humans
  • Lamins / metabolism*
  • Nuclear Envelope / metabolism
  • Progeria / metabolism*
  • Progeria / pathology
  • Werner Syndrome / metabolism*
  • Werner Syndrome / pathology


  • Lamins