Animal Models of Huntington’s Disease

Huntington's disease (HD) is a neurological disorder caused due to mutation in the dominant IT15 gene. It is a “trinucleotide repeat” disorder caused by an increase in the number of CAG repeats in the HD gene. Progressive cell death in the cortex and striatum accompanied by a decline in cognitive, motor, and psychiatric functions are the disease's characteristics. Various animal models for HD have been developed to provide insight into disease pathology and study therapeutic strategies outcomes. Previous HD studies are primarily based on toxin-induced models to learn mitochondrial dysfunction and cell death induced by excitotoxicity seen in the HD brain. The discovery of the huntingtin mutation in 1993 resulted in creating new models that introduce a similar genetic defect in animals and then studied the disease's pathophysiology. Various Genetic models are developed to date. Invertebrate models of HD: Caenorhabditis elegans and Drosophila melanogaster models; rodent models, and some transgenic large animal models are discussed here. Each model has its advantages and limitations. The researcher must decide which model to use depending on the study's type and requirements.

Keywords: Excitotoxicity, Genetic models, Huntington`s disease, Mitochondrial dysfunction.