Defective responses to DNA single- and double-strand breaks in spinocerebellar ataxia

Failure to maintain the integrity of DNA/chromatin can result in genome instability and an increased risk of cancer. The description of a number of human genetic disorders characterised not only by cancer predisposition but by a broader phenotype including neurodegeneration suggests that maintaining genome stability is also important for preserving post-mitotic neurons. The identification of genes associated with other neurodegenerative disorders provides further evidence for the importance of DNA damage response and DNA repair genes in protecting against neurodegeneration. This theme is further developed in this review.