DNA double-strand breaks (DSBs) are major threats to the genomic integrity
of cells. If not taken care of properly, they can cause chromosome fragment
ation, loss and translocation, possibly resulting in carcinogenesis. Upon D
SB formation, cell-cycle checkpoints are triggered and multiple DSB repair
pathways can be activated. Recent research on the Nijmegen breakage syndrom
e, which predisposes patients to cancer, suggests a direct link between act
ivation of cell-cycle checkpoints and DSB repair. Furthermore, the biochemi
cal activities of proteins involved in the two major DSB repair pathways, h
omologous recombination and DNA end-joining, are now beginning to emerge. T
his review discusses these new findings and their implications for the mech
anisms of DSB repair.