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Our Huntington’s Disease Models
120 CAG R6/2 Mouse Model
Our partnership with the CHDI Foundation has empowered us to extensively study the R6/2 HD mouse model. This model expresses the N-terminal portion of the huntingtin protein with approximately 120 CAG repeats.
zQ175 Knock-In Model
Notably, the zQ175 model exhibits a slow progression of Huntington’s disease-related abnormalities, including decreased striatal volume, motor impairment, and basal ganglia circuit abnormalities. Through studying this model, we glean valuable insights into the underlying mechanisms of Huntington’s disease and explore potential therapeutic interventions.
The zQ175 knock-in model shows a progressive motor and cognitive decline consistent with other HD models. Specifically, this model exhibits age-related decline in body weight, reduced survival, motor deficits, and apathy, which are more pronounced in zQ175 homozygous mice.