Animal Models of Huntingtons Disease

Huntington’s disease (HD) is an inherited, progressive neurodegenerative disorder transmitted by a single autosomal dominant gene. The symptomology of the disease was first described by George Huntington in 1872 as consisting of a progressive dementia coupled with bizarre uncontrollable movements and abnormal postures. HD is found in nearly all ethnic and racial groups with slight variations in prevalence rates. Overall, the prevalence rate of HD in the US is approx 50/1,000,000 (Reed and Chandler, 1958 ; Sanberg and Coyle, 1984 ). Although HD may occur during the juvenile years (Korenyi and Whittier, 1973 ); the manifestation of the disorder typically occurs in middle life, about 35–45 years of age. From the time of onset, an intractable course of mental deterioration and progressive motor abnormalities begins with death usually occurring within 15 years.