title: Correction of the DNA repair defect in Fanconi anemia complementation groups A and D cells.
abstract: We have previously isolated from Fanconi anemia, complementation groups A (FA-A) and D (FA-D) cells, a DNA endonuclease complex which is defective in its ability to incise DNA containing interstrand cross-links produced by psoralen plus UVA light. The repair capabilities of the FA complexes, compared with those of the corresponding normal complex, have now been examined using two types of complementation analysis. First, introduction of the normal complex, by electroporation, into 8-methoxypsoralen (8-MOP) plus UVA treated FA-A and FA-D cells resulted in correction of their repair defect, determined by measuring repair-related unscheduled DNA synthesis (UDS). The FA-A and FA-D complexes could similarly complement the repair defect in each others' cells, but not in their own. Second, mixing the normal with the FA-A and FA-D complexes, or the FA-A with the FA-D complex, in a cell-free system resulted in correction of the defect in ability of these FA complexes to incise damaged DNA. These results indicate that the normal complex contains the proteins needed to correct the DNA repair defect in FA-A and FA-D cells and that the FA-A and FA-D complexes contain the protein needed to complement the repair defect in each other.
authors.affiliation: Department of Pathology and Laboratory Medicine, UMDNJ-New Jersey Medical School, Newark 07103, USA. mlambert@umdnj.edu
authors.forename: M W
authors.initials: MW
authors.lastname: Lambert
authors.forename: G J
authors.initials: GJ
authors.lastname: Tsongalis
authors.forename: W C
authors.initials: WC
authors.lastname: Lambert
authors.forename: D D
authors.initials: DD
authors.lastname: Parrish
mesh_terms: D002461:Cell Line, Transformed; D002474:Cell-Free System / Q000201:enzymology; D004247:DNA / Q000096:biosynthesis; D004260:DNA Repair; D018274:Electroporation; D004706:Endodeoxyribonucleases / Q000235:genetics; D005199:Fanconi Anemia / Q000201:enzymology / Q000235:genetics*; D005816:Genetic Complementation Test; D006801:Humans; D008214:Lymphocytes / Q000201:enzymology
chemical_list: D004247:DNA; D004706:Endodeoxyribonucleases