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Rodent Models of Huntington's Disease: An Overview

Gallilio Fernadez

Huntington's Disease (HD) is a progressive neurodegenerative disorder characterized by motor dysfunction, cognitive decline, and psychiatric symptoms. It is caused by a genetic mutation that leads to an abnormal expansion of CAG repeats in the Huntingtin (HTT) gene, resulting in an expanded polyglutamine tract in the huntingtin protein. This mutation induces a cascade of cellular dysfunctions culminating in the death of specific neuronal populations, predominantly in the striatum and cortex. While human studies provide critical insights, animal models, particularly rodent models, have been invaluable for understanding the pathophysiology of HD and for developing therapeutic strategies.