Role of oxidative DNA damage in mitochondrial dysfunction and Huntington¡¯s disease pathogenesis

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• 作者：Sylvette Ayala-Pe?a
• 关键词：AP ; apurinic/apyrimidinic ; ARE ; antioxidant response element ; ATM ; ataxia telangiectasia mutated kinase ; ATR ; Rad3-related kinase ; BER ; base excision repair ; FEN1 ; flap endonuclease 1 ; HD ; Huntington&rsquo ; s disease ; MMR ; mismatch repair ; Nrf2 ; NF-E2-rela
• 刊名：Free Radical Biology and Medicine
• 出版年：2013
• 出版时间：September, 2013
• 年：2013
• 卷：62
• 期：Complete
• 页码：102-110
• 全文大小：701 K

文摘

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.