Involvement of the Fanconi anemia protein FA-C in repair processes of oxidative DNA damages.
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.
Lackinger
D
D
Institute of Biochemistry, Free University Berlin, Germany.
Ruppitsch
W
W
Ramirez
M H
MH
Hirsch-Kauffmann
M
M
Schweiger
M
M
D018797:Cell Cycle Proteins; D002460:Cell Line; D015500:Chloramphenicol O-Acetyltransferase; D004249:DNA Damage; D004260:DNA Repair; D004278:DNA, Superhelical / Q000378:metabolism; D004268:DNA-Binding Proteins; D004587:Electrophoresis, Agar Gel; D005199:Fanconi Anemia / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D051856:Fanconi Anemia Complementation Group Proteins; D017930:Genes, Reporter; D006801:Humans; D008214:Lymphocytes / Q000378:metabolism; D009687:Nuclear Proteins; D018384:Oxidative Stress; D010957:Plasmids / Q000378:metabolism; D011196:Potassium Permanganate / Q000494:pharmacology; D011506:Proteins / Q000235:genetics / Q000502:physiology*; D014162:Transfection
D018797:Cell Cycle Proteins; D004278:DNA, Superhelical; D004268:DNA-Binding Proteins; D051856:Fanconi Anemia Complementation Group Proteins; D009687:Nuclear Proteins; D011506:Proteins; D011196:Potassium Permanganate; D015500:Chloramphenicol O-Acetyltransferase