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Involvement of the Fanconi anemia protein FA‐C in repair processes of oxidative DNA damages
Author(s) -
Lackinger Dagmar,
Ruppitsch Werner,
Ramirez Maria Helena,
Hirsch-Kauffmann Monica,
Schweiger Manfred
Publication year - 1998
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/s0014-5793(98)01442-2
Subject(s) - fanconi anemia , complementation , dna repair , dna damage , lymphoblast , microbiology and biotechnology , oxidative phosphorylation , biology , chemistry , biochemistry , dna , cell culture , gene , mutant , genetics
Fanconi anemia (FA) is an autosomal recessive disorder characterized by skeletal abnormalities, pancytopenia and a marked predisposition to cancer. FA cells exhibit chromosomal instability and hypersensitivity towards oxygen and cross‐linking agents such as diepoxybutane and mitomycin C. An increased level of reactive oxygen intermediates and an elevation of 8‐oxoguanine in FA cells point to a defective oxygen metabolism in FA cells. We investigated the repair activity of oxidatively damaged DNA in lymphoblastoid cells from FA patients of complementation groups A–E. The repair activity for oxidatively damaged DNA was significantly reduced in lymphoblastoid cell lines of complementation groups B–E. Complementation of the FA‐C cell line with the wild type FA‐C gene restored the repair activity to normal. This indicates that the FA‐C protein participates in the repair of oxidatively damaged DNA.