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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.

Type

Journal article

Journal

Environ Mol Mutagen

Publication Date

2001

Volume

38

Pages

227 - 234

Keywords

Cell Line, DNA Helicases, DNA Repair, DNA, Mitochondrial, Exodeoxyribonucleases, Humans, Mutagenesis, RecQ Helicases, Werner Syndrome, Werner Syndrome Helicase