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Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease

M B Bogdanov1, O A Andreassen, A Dedeoglu

  • 1Department of Neurology and Neuroscience, Weill Medical College of Cornell University and New York Presbyterian Hospital, New York, NY 10021, USA.

Journal of Neurochemistry
|December 26, 2001
PubMed

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Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...