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Chunk #23 — Use of iPSCs to model neurodegenerative diseases — iPSCs and huntington's disease

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Modeling Human Neurological and Neurodegenerative Diseases: From Induced Pluripotent Stem Cells to Neuronal Differentiation and Its Applications in Neurotrauma.
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Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by a CAG trinucleotide repeat expansion in the huntingtin gene that generates long polyglutamine stretches in the encoded huntingtin protein (HTT). This leads to a massive loss of medium spiny neurons in the striatum and loss of neurons in the cortex as the disease progresses. Personality changes, weight loss, involuntary movements and dementia are the principal changes mostly developed among the people carrying the huntingtin gene mutation.