DNA repair and mutagenesis in Werner syndrome

Werner syndrome (WS) is the hallmark premature aging syndrome in which the patients appear much older than their actual chronological age. The disorde r is associated with significantly increased genome instability and with tr anscriptional deficiencies. There has been some uncertainty about whether W S cells are defective in DNA repair. We thus examined repair in vitro in nu clear and mitochondria( DNA. Whereas cellular studies so far do not show si gnificant DNA repair deficiencies, biochemical studies with the Werner prot ein clearly indicate that it plays a role in DNA repair. Published 2001 Wil ey-Liss, Inc.