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Huntington’s disease (HD) is a neurodegenerative disorder with an autosomal dominant expression pattern and typically a late-onset appearance. HD is a movement disorder with a heterogeneous phenotype characterized by involuntary dance-like gait, bioenergetic deficits, motor impairment, and cognitive and psychiatric deficits. Compelling evidence suggests that increased oxidative stress and mitochondrial dysfunction may underlie HD pathogenesis. However, the exact mechanisms underlying mutant huntingtin-induced neurological toxicity remain unclear. The objective of this paper is to review recent literature regarding the role of oxidative DNA damage in mitochondrial dysfunction and HD pathogenesis.• Oxidative DNA damage in dysfunctional mitochondrial bioenergetics associated with the pathogenesis of Huntington’s disease.• Oxidative damage to DNA and to mitochondria in Huntington’s disease.• Oxidative DNA repair of both nuclear and mitochondrial genomes in Huntington’s disease.