Telomere ResQue and preservation--roles for the Werner syndrome protein and other RecQ helicases.
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.
Opresko
Patricia L
PL
Department of Environmental and Occupational Health, University of Pittsburgh Graduate School of Public Health, Bridgeside Pt., Pittsburgh, PA 15219, United States. plo4@pitt.edu
D000818:Animals; D004261:DNA Replication; D005090:Exodeoxyribonucleases; D006801:Humans; D051379:Mice; D053484:RecQ Helicases / Q000235:genetics / Q000502:physiology*; D016615:Telomere / Q000737:chemistry / Q000378:metabolism*; D014898:Werner Syndrome / Q000201:enzymology* / Q000235:genetics; D000071657:Werner Syndrome Helicase
D005090:Exodeoxyribonucleases; D053484:RecQ Helicases; C098785:WRN protein, human; D000071657:Werner Syndrome Helicase; C499253:Wrn protein, mouse