Many inherited human DNA repair deficiency syndromes involve pronounced neurological dysfunction, although the DNA lesions responsible are generally unknown. A new study shows that the phosphodiesterase TDP2 has a key role in protecting the nervous system by preventing DNA breaks induced by aberrant topoisomerase II activity.
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McKinnon, P. TDP2 keeps the brain healthy. Nat Genet 46, 419–421 (2014). https://doi.org/10.1038/ng.2967