Deficient DNA repair in the human progeroid disorder, Werner syndrome.
The study of how DNA repair mechanisms change with aging is central to our understanding of the aging process. Here, I review the molecular functions of a key aging protein, Werner protein (WRN), which is deficient in the premature aging disorder, Werner syndrome (WS). This protein plays a significant role in DNA repair, particularly in base excision repair and in recombination. WRN may be a key regulatory factor in these processes and may also play a role in coordinating them. WRN belongs to the RecQ helicase family of proteins, often referred to as the guardians of the genome. These proteins appear to integrate with the more classic DNA repair pathways and proteins.
Bohr
Vilhelm A
VA
Laboratory of Molecular Gerontology, National Institute on Aging, NIH, 5600 Nathan Shock Dr., Baltimore, MD 21224, USA. vbohr@nih.gov
D004265:DNA Helicases / Q000502:physiology; D004260:DNA Repair / Q000235:genetics* / Q000502:physiology; D005090:Exodeoxyribonucleases; D006801:Humans; D053484:RecQ Helicases; D016615:Telomere; D014898:Werner Syndrome / Q000235:genetics*; D000071657:Werner Syndrome Helicase
D005090:Exodeoxyribonucleases; D004265:DNA Helicases; D053484:RecQ Helicases; C098785:WRN protein, human; D000071657:Werner Syndrome Helicase