@entity11807 ( @entity11807 ) is a multigenic @entity74 resulting in @entity2131 and increased @entity5 susceptibility. Cells from @entity11807 @entity1 and @entity19 models are sensitive to DNA interstrand crosslinks (ICLs) and @entity11807 @entity19 are moderately sensitive to ionizing radiation (IR). Both kinds of damage induce DNA double strand breaks (DSBs). To date, nine genes in 11 complementation groups have been identified; however, the precise function of the @entity11807 pathway remains unclear. Many of the proteins form a nuclear complex necessary for the mono-ubiquitination of the downstream protein, @entity27797 . To further investigate the role of the @entity11807 pathway in repair of DSBs, we generated @entity27797 (-/-)/Prkdc(sc/sc) double mutant @entity19 . Prkdc(sc/sc) mutant @entity19 have a defect in non-homologous end joining (NHEJ) and are sensitive to IR-induced DNA damage. Double mutant animals and primary cells were more sensitive to IR than either single mutant, suggesting that @entity27797 operates in DSB repair pathway distinct from NHEJ. @entity27797 (-/-)/Prkdc(sc/sc) double mutant cells were also more sensitive to DSBs generated by a restriction endonuclease. The role of @entity27797 in DSB repair may account for the moderate sensitivity of @entity11807 cells to irradiation and @entity11807 cells sensitivity to ICLs that are repaired via a DSB intermediate.

XXXX functions in a double strand break repair pathway that is distinct from non-homologous end joining.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity27797 :: ('211651(Tax:10090)', 'Gene') :: ['Fancd2'] @entity5 :: ('MESH:D009369', 'Disease') :: ['cancer'] @entity19 :: ('10090', 'Species') :: ['mice', 'mouse'] @entity74 :: ('MESH:D030342', 'Disease') :: ['recessive disease'] @entity2131 :: ('MESH:C536572', 'Disease') :: ['bone marrow failure'] @entity11807 :: ('MESH:D005199', 'Disease') :: ['Fanconi anemia', 'FA']
@entity27797 :: (211651(Tax:10090),Gene) :: ['Fancd2']
