This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Huntington's disease (HD) is an autosomal, dominant, neurodegenerative disorder that results from an unstable expansion of the trinucleotide repeat CAG in the gene IT-15, or huntington. HD has a prevalence of 5-10 per 100,000 population. In the United States there are approximately 30,000 individuals with clinical features of HD and another 200,000 at risk for HD. The clinical features of HD usually emerge in adulthood (mean age of 37 years) with chorea, disorders of voluntary movement, intellectual dysfunction, and psychiatric symptoms. HD is relentless, leading to functional disability and death over a period of 10-30 years. The Neurobiological Predictors of Huntington's Disease (PREDICT-HD) study will conduct an intensive examination in persons with known gene status. The primary aim in presymptomatic persons is to determine whether a specific therapeutic intervention can safely delay the age of disease onset.
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