Editors' ChoiceDNA Repair

Righting Repair Pathways

Sci. Signal.  13 Jul 2010:
Vol. 3, Issue 130, pp. ec216
DOI: 10.1126/scisignal.3130ec216

The genetic disease Fanconi anemia (FA) results from mutations in a series of genes involved in a DNA repair pathway that helps process the damage caused by erroneous chemical cross-links between the two strands of the DNA double helix. The double-stranded breaks in DNA that arise from such cross-links can be repaired in an error-free manner or through an error-prone repair pathway. Pace et al. show that the FA pathway can drive repair through the error-free pathway. The FA FANCC gene shows a genetic interaction with a component of the error-prone repair pathway, Ku70, inhibiting its action and thereby promoting the error-free pathway.

P. Pace, G. Mosedale, M. R. Hodskinson, I. V. Rosado, M. Sivasubramaniam, K. J. Patel, Ku70 corrupts DNA repair in the absence of the Fanconi anemia pathway. Science 329, 219–223 (2010). [Abstract] [Full Text]