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DNA repair and mutagenesis in Werner syndrome
Author(s) -
Bohr Vilhelm A.,
Souza Pinto Nadja,
Nyaga Simon G.,
Dianov Grigory,
Kraemer Kenneth,
Seidman Michael M.,
Brosh Robert M.
Publication year - 2001
Publication title -
environmental and molecular mutagenesis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 87
eISSN - 1098-2280
pISSN - 0893-6692
DOI - 10.1002/em.1076
Subject(s) - werner syndrome , genome instability , dna repair , premature aging , biology , mutagenesis , cockayne syndrome , genetics , mitochondrial dna , dna , nuclear dna , dna damage , mutation , microbiology and biotechnology , nucleotide excision repair , gene , helicase , rna
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. Environ. Mol. Mutagen. 38:227–234, 2001. Published 2001 Wiley‐Liss, Inc.