Q175 Knock-in Mouse Model of Huntington’s Disease in Humans

The Q175 knock-in mouse contains human mHtt allele with the expanded CAG repeat (~179 repeats) within the native mouse huntingtin gene. Thus, this animal model is representative of Huntington’s disease in humans from a genetic aspect. Both homo- and heterozygous Q175 mice exhibit first signs of motor symptoms early, from 3-4 months of age, and behavioral deficits are accompanied by marked brain atrophy and brain metabolite changes by 8 months.

Charles River conducts contract studies in Q175 knock-in mice to test the efficacy of novel therapeutics for treating Huntington’s disease. The Q175 mouse shows robust, gene dose-dependent, progressive and early-onset alterations in the validated endpoint assays. All studies can be customized according to client needs.

Study Components

  • Motor deficits
    • Open field test
    • Rear climbing
  • Cognitive deficits
    • Procedural two-choice swim test
  • Neurological index
  • Imaging
    • In vivo MRI for brain volumetry (whole brain, cortex, striatum)
    • In vivo 1H-MRS of the striatum metabolic profile
  • Biomarkers
  • Various options in mRNA and protein detection assays