Department of Pharmacology and Toxicology, University of Puerto Rico Medical Sciences Campus, P.O. Box 365067, San Juan, Puerto Rico 00936-5067.
Free Radic Biol Med. 2013 Sep;62:102-110. doi: 10.1016/j.freeradbiomed.2013.04.017. Epub 2013 Apr 18.
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.
亨廷顿病（HD）是一种具有常染色体显性表达模式且通常表现为迟发性的神经退行性疾病。HD 是一种运动障碍，其异质性表型的特征是不自主的舞蹈样步态、生物能量缺陷、运动障碍以及认知和精神障碍。强有力的证据表明，氧化应激和线粒体功能障碍的增加可能是 HD 发病机制的基础。然而，突变亨廷顿蛋白诱导的神经毒性的确切机制仍不清楚。本文的目的是回顾最近关于氧化 DNA 损伤在线粒体功能障碍和 HD 发病机制中的作用的文献。
