DNA double-strand breaks (DSBs) are common events that were recognized as one of the
most toxic lesions in eukaryotic cells. DSBs are widely involved in many physiological processes such as
V(D)J recombination, meiotic recombination, DNA replication and transcription. Deregulation of DSBs
has been reported in multiple diseases in human beings such as the neurodegenerative diseases, with
which the underlying mechanisms are needed to be illustrated. Here, we reviewed the recent insights
into dysfunction of DSB formation and repair contributing to the pathogenesis of neurodegenerative
disorders including Alzheimer’s disease (AD), amyotrophic lateral sclerosis (ALS), Huntington’s disease
(HD) and ataxia telangiectasia (A-T).