Abstract
Huntington’s disease (HD) is an autosomal dominant, hyperkinetic movement disorder, and progressive neurodegenerative disorder characterized by choreiform abnormal movements, cognitive deficits, psychiatric manifestations, emotional disturbances, and dementia associated with progressive striatal atrophy. The disorder was first described in 1872 by Dr. George Huntington in his article titled “On Chorea.” Dr. Huntington entitled the disease “Hereditary Chorea,” whereas observations of some cases which do not present with chorea later led to the acceptance of the “Huntington’s Disease” label. The HD gene (IT15) is sited on the short arm of chromosome 4, and the normal number of CAG (glutamine) repeats is expanded (generally >40). Genetic mutation in HD gene results in an expanded polyglutamine stretch in the NH2 terminus of huntingtin protein (HTT).
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Kaur, N., Jamwal, S., Kaur Gill, H., Bansal, P.K. (2017). Animal Models of Huntington’s Disease. In: Bansal, P., Deshmukh, R. (eds) Animal Models of Neurological Disorders. Springer, Singapore. https://doi.org/10.1007/978-981-10-5981-0_4
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