1. DNA repair and mutagenesis in Werner syndrome.
- Author
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Bohr VA, Souza Pinto N, Nyaga SG, Dianov G, Kraemer K, Seidman MM, and Brosh RM Jr
- Subjects
- Cell Line, DNA Helicases genetics, DNA, Mitochondrial genetics, Exodeoxyribonucleases, Humans, RecQ Helicases, Werner Syndrome Helicase, DNA Repair, Mutagenesis, Werner Syndrome genetics
- Abstract
Werner syndrome (WS) is the hallmark premature aging syndrome in which the patients appear much older than their actual chronological age. The disorder is associated with significantly increased genome instability and with transcriptional deficiencies. There has been some uncertainty about whether WS cells are defective in DNA repair. We thus examined repair in vitro in nuclear and mitochondrial DNA. Whereas cellular studies so far do not show significant DNA repair deficiencies, biochemical studies with the Werner protein clearly indicate that it plays a role in DNA repair.
- Published
- 2001
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