期刊
MECHANISMS OF AGEING AND DEVELOPMENT
卷 129, 期 1-2, 页码 79-90出版社
ELSEVIER IRELAND LTD
DOI: 10.1016/j.mad.2007.10.007
关键词
Werner syndrome; RecQ helicase; telomere; DNA replication and repair
资金
- NIEHS NIH HHS [ES015052-01] Funding Source: Medline
- NATIONAL INSTITUTE OF ENVIRONMENTAL HEALTH SCIENCES [R01ES015052] Funding Source: NIH RePORTER
Werner syndrome is an autosomal recessive disorder resulting from loss of function of the RecQ helicase, WRN protein. WS patients prematurely develop numerous clinical symptoms and diseases associated with aging early in life and are predisposed to cancer. WRN protein and many other RecQ helicases in general, seem to function during DNA replication in the processing of stalled replication forks. Genetic, cellular and biochemical evidence support roles for WRN in proper replication and repair of telomeric DNA, and indicate that telomere dysfunction contributes to the WS disease pathology. (C) 2007 Elsevier Ireland Ltd. All rights reserved.
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