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 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.
Bohr
V A
VA
Laboratory of Molecular Gerontology, National Institute on Aging, National Institutes of Health, Baltimore, Maryland 21224, USA. vbohr@nih.gov
Souza Pinto
N
N
Nyaga
S G
SG
Dianov
G
G
Kraemer
K
K
Seidman
M M
MM
Brosh
R M
RM
D002460:Cell Line; D004265:DNA Helicases / Q000235:genetics; D004260:DNA Repair; D004272:DNA, Mitochondrial / Q000235:genetics; D005090:Exodeoxyribonucleases; D006801:Humans; D016296:Mutagenesis; D053484:RecQ Helicases; D014898:Werner Syndrome / Q000235:genetics*; D000071657:Werner Syndrome Helicase
D004272:DNA, Mitochondrial; D005090:Exodeoxyribonucleases; D004265:DNA Helicases; D053484:RecQ Helicases; C098785:WRN protein, human; D000071657:Werner Syndrome Helicase