Huntington and the pathogenesis of Huntington’s disease (HD)
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medical studies
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published 01/08/2008
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SummaryHuntington’s disease (HD) is a progressive neurodegenerative disorder with an established genetic origin. Adult onset HD is characterized by motor dysfunction, cognitive decline and psychiatric disturbance (Ranen et al., 1993 and Harper, 1996). Initially symptoms include depression, irritable mood, and some minor involuntary movements (Young et al., 1986 and Harper, 1996). As the disease progresses, chorea becomes more evident. The most prominent characteristics of HD include a profound loss of neurons in the caudate nucleus and putamen, which together comprise the corpus striatum within the basal ganglia (Ross, 1995). HD is caused by an elongation of a polyglutamine tract in the N-terminal of the huntingtin gene placing it into the trinucleotide repeat disorders family of neurologic diseases.
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