期刊
JOURNAL OF CELL SCIENCE
卷 120, 期 5, 页码 713-721出版社
COMPANY BIOLOGISTS LTD
DOI: 10.1242/jcs.03397
关键词
Werner Syndrome; telomere; DNA damage; aging; cancer
类别
资金
- NATIONAL CANCER INSTITUTE [R01CA129037] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE ON AGING [R01AG028888] Funding Source: NIH RePORTER
- NCI NIH HHS [R01 CA129037] Funding Source: Medline
- NIA NIH HHS [R01 AG028888] Funding Source: Medline
Werner Syndrome (WS) is a premature aging syndrome characterized by early onset of age-related pathologies and cancer. Since WS is due to a single gene defect, it has attracted much interest from researchers seeking to understand pathways that contribute to cancer and aging at cellular and molecular levels. The protein mutated in WS, WRN, appears to play a major role in genome stability, particularly during DNA replication and telomere metabolism. Much of the pathophysiology associated with WS, including the rapid onset of cellular senescence, early cancer onset and premature aging, can be attributed to defect in telomere maintenance. Recent genetic evidence from the mTerc(-/-) Wrn(-/-) mouse demonstrates that mice with critically shortened telomeres display aging phenotypes reminiscent of human WS, further reinforcing the notion that telomere dysfunction is required for the manifestation of aging pathophysiologies in the setting of WRN deficiency.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据