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B
roken chromosomes pose a serious threat to cell sur-
vival. The presence of an unrepaired double-strand
break (DSB) will trigger the DNA-damage response sys-
tems of a cell to arrest its progression through the cell
cycle and, sometimes, to cause apoptotic cell death. But
even if a cell with an unrepaired DSB continues to divide,
the broken chromosome fragments will mis-segregate and
be degraded, producing aneuploidy.
In response to this threat, cells have elaborated an
impressive arsenal of DNA-repair pathways. There are
two general types of repair: homologous recombination
(HR) and nonhomologous end-joining (NHEJ). These two
processes are in competition with each other and one
focus of this review is to examine the way that this compe-
tition is regulated. But the cell’s options are far more com-
plex than simply electing to employ HR or NHEJ. There
are several types of homologous repair: gene conversion,
break-induced replication and single-strand annealing
(reviewed in Ref. 1). Similarly, there are also several alter-
native end-joining mechanisms
2,3
. Moreover, even once a
process such as gene conversion is initiated, there are
additional genetically regulated decisions in choosing
among alternative homologous templates to carry out
repair. How does the cell decide to use a template on a sister
chromatid, on a homologous chromosome or at an ectopic
site? Moreover, how are these choices tied to the cell’s
DNA damage-sensing checkpoints?
This review surveys the impressive recent progress in
delineating the different mechanisms of homologous and
nonhomologous repair and the way in which they all com-
pete in repairing DSBs. The emphasis will be on what has
been learned in the best-studied organism, Saccharomyces
cerevisiae, but I also discuss studies in other model eukary-
otic systems and in mammalian cells.
Homologous recombination mechanisms
The three major types of HR all begin in the same way, as
the ends of the DSB are resected by 5' to 3' exonucleases
or by a helicase coupled to an endonuclease, to produce
long, 3'-ended single-stranded DNA tails (Fig. 1).
Single-strand annealing
In the simplest process (Fig. 1d), resection exposes com-
plementary regions of homologous sequences originally
flanking the DSB, creating a deletion by single-strand
annealing (SSA). SSA will occur with as little as 30 bp of
homology, although it is much more efficient with
200–400 bp (Ref. 44).
Double-strand chromosome breaks can arise in a number of ways, by ionizing radiation, by spontaneous
chromosome breaks during DNA replication, or by the programmed action of endonucleases, such as in
meiosis. Broken chromosomes can be repaired either by one of several homologous recombination
mechanisms, or by a number of nonhomologous repair processes. Many of these pathways compete actively for
the repair of a double-strand break. Which of these repair pathways is used appears to be regulated
developmentally, genetically and during the cell cycle.
Partners and pathways
repairing a double-strand break
James E. Haber
haber@brandeis.edu
Rosentiel Basic Medical
Sciences Research
Center, MS 029 Brandeis
University, 415 South
Street, Waltham, MA
02454-9110, USA.
0168-9525/00/$ – see front matter © 2000 Elsevier Science Ltd. All rights reserved. PII: S0168-9525(00)02022-9