Aging and nuclear organization: lamins and progeria |
| |
Authors: | Mounkes Leslie C Stewart Colin L |
| |
Affiliation: | Cancer and Developmental Biology Laboratory, National Cancer Institute at Frederick, National Institutes of Health, PO Box B, Frederick, Maryland 21702, USA. |
| |
Abstract: | 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. |
| |
Keywords: | |
本文献已被 PubMed 等数据库收录! |
|