potassium-permanganate and Fanconi-Anemia

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.

Topics: Cell Cycle Proteins; Cell Line; Chloramphenicol O-Acetyltransferase; DNA Damage; DNA Repair; DNA-Binding Proteins; DNA, Superhelical; Electrophoresis, Agar Gel; Fanconi Anemia; Fanconi Anemia Complementation Group Proteins; Genes, Reporter; Humans; Lymphocytes; Nuclear Proteins; Oxidative Stress; Plasmids; Potassium Permanganate; Proteins; Transfection