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- W2505729255 abstract "Publisher Summary Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder characterized by movement disorders, cognitive deterioration, and psychiatric symptoms. It is caused by a (CAG)n n trinucleotide repeat expansion close to the 5' end of a gene encoding the huntingtin protein. The pathology of HD is characterized by a selectivity of neuronal loss with greatest severity in the striatum and the deeper layers of the cerebral cortex. The disease is associated with the formation of intraneuronal inclusions or aggregates. The role of these aggregates, which can be found in both the nucleus and cytoplasm, is controversial and the debates are reviewed in this chapter, drawing on experimental data from animal and cellular models of HD and related polyglutamine diseases. The chapter considers the possibility that early changes in cAMP-mediated transcription may play a role in the early changes seen in HD. The potential roles of excitotoxicity and mitochondrial dysfunction in disease pathogenesis are discussed and form a backdrop for a review of some of the approaches that are being used as experimental therapies for this currently incurable disease. Finally, the chapter reviews the genetics and biology of some diseases that can mimic HD symptomatology." @default.
- W2505729255 created "2016-08-23" @default.
- W2505729255 creator A5008236816 @default.
- W2505729255 date "2003-01-01" @default.
- W2505729255 modified "2023-10-14" @default.
- W2505729255 title "Molecular Biology of Huntington's Disease (HD) and HD-Like Disorders" @default.
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