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Papers In Press, published online ahead of print May 14, 2003
Pharmacology, University of Minnesota, Minneapolis, MN 55455
Corresponding Author: dona0054{at}tc.umn.edu
Fibroblasts from patients with Fanconi anemia (FA) display genomic instability, hypersensitivity to DNA crosslinking agents, and deficient DNA end-joining. Fibroblasts from two FA patients of unidentified complementation group also have significantly increased cellular homologous recombination (HR) activity. Results described herein show that HR activity levels in patient-derived FA fibroblasts of groups A, C, and G were 10-fold greater than those seen in normal fibroblasts. In contrast, HR activity in group D2 fibroblasts was identical to that of normal cells. Western blot analysis revealed that RAD51 protein was elevated 10-fold above normal in group A, C, and G fibroblasts, but was not altered in D2 fibroblasts. HR activity levels in these former cells could be restored to near-normal levels by electroporation with an anti-RAD51 antibody, while similar treatment of normal and complementation group D2 fibroblasts had no effect. These findings are consistent with a model in which FA proteins function to coordinate DNA double-strand break repair activity by regulating both recombinational and non-recombinational DNA repair. Interestingly, whereas positive regulation of DNA end-joining requires the combined presence of all FA proteins thus far tested, suppression of HR, which is minimally dependent on the FANCA, FANCC and FANCG proteins, does not require FANCD2.
J. Biol. Chem, 10.1074/jbc.M213251200
Submitted on December 30, 2002
Revised on May 10, 2003
Accepted on May 13, 2003
Deficient regulation of DNA double-strand break repair in fanconi anemia fibroblasts
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