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N Am J Med Sci (Boston). 2010;3(4):205-207.

WRN protein and Werner syndrome.

Author information

  • 1Department of Medical & Research Technology, Department of Pathology, School of Medicine, University of Maryland, AHB 405A, 100 Penn Street, Baltimore, MD 21201.

Abstract

Werner syndrome is an autosomal recessive disorder associated with premature aging and cancer predisposition. Cells from Werner syndrome patients show increased genomic instability and are hypersensitive to DNA damage agents. Werner syndrome is caused by mutations of the WRN gene. WRN protein is a member of RecQ DNA helicase family. It not only contains a conserved 3'-5' helicase domain as other members of the RecQ family but also contains a unique 3'-5' exonuclease domain. WRN recognizes specific DNA structures as substrates which are intermediates of DNA metabolism. WRN interacts with many other proteins, which function in telomere maintenance, DNA replication, and DNA repair through different pathways.

PMID:
22180828
[PubMed]
PMCID:
PMC3237395
Free PMC Article
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