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Bioessays. 2000 Oct;22(10):894-901.

The Werner syndrome protein: an update.

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  • 1Department of Pathology, Box 357470, HSB K-543. University of Washington, 1959 NE Pacific Ave., Seattle, WA 98195-7470, USA. picard@u.washington.edu

Abstract

Progeria and progeroid syndromes are characterized by the earlier onset of complex senescent phenotypes. WRN was originally identified as a gene responsible for Werner syndrome (WS; "Progeria of Adults"). The WRN gene product has RecQ-type helicase domains in the central region of the protein. Subsequent studies also revealed that the WRN protein displays exonuclease activity and acts as a transcriptional activation factor. These biochemical studies, combined with cell biological studies, suggested that this protein is likely to be involved in the response to DNA damage during replication, as well as recombination and transcription processes. However, the precise molecular mechanisms by which mutations in WRN cause the WS phenotype remain unknown. Recent progress in the understanding of the WRN protein and its implication in the normal aging process are discussed.

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