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Leverage the world’s largest collections of mouse models of Huntington’s disease and more than 80 years of neurobiology research experience.
This table summarizes the differences between important mouse models for Huntington's disease to help you easily find the right strain for your research.
| Strain Name | Common Name | Molecular Mutation | Phenotype | Survival |
|---|---|---|---|---|
| R6/2 | Mutant human huntingtin fragment containing ~1 kb of 5' UTR region and a polyglutamate-repeat (CAG) expansion of 160 +/- 5, and 262 bp of intron 1 | Neuronal intranuclear inclusions, choreiform-like and involuntary stereotypic movements, tremors, and epileptic seizures by 9-11 weeks | Premature death between 10 to 13 weeks of age | |
| R6/2 substrain | Mutant human huntingtin fragment containing ~1 kb of 5' UTR region and a polyglutamate-repeat (CAG) expansion of 120 +/- 5, and 262 bp of intron 1 | Neuronal intranuclear inclusions, choreiform-like and involuntary stereotypic movements, tremors, and epileptic seizures by 14-16 weeks | Premature death between 15 to 16 weeks of age | |
| R6/1 | Mutant human huntingtin fragment containing ~1 kb of 5' UTR region and exon 1 containing a 115-150 polyglutamate-repeat (CAG), and 262 bp of intron 1 | Neuronal intranuclear inclusions, choreiform-like and involuntary stereotypic movements, tremors, and epileptic seizures by 15-21 weeks | Premature death between 32 to 40 weeks of age | |
| HD-N171-82Q-81 | Mouse prion protein promoter driving an N-terminally truncated human huntingtin cDNA that encodes 82 glutamines and encompasses the first 171 amino acids | Neuronal intranuclear inclusions, tremors, hypokinesis and lack of coordination after 2 months of age | Premature death between 16 to 30 weeks of age | |
| YAC128 | Yeast artificial chromosome containing an endogenous human promoter driving a full-length human huntingtin gene modified with a 128 CAG repeat expansion | Hyperkinesis beginning at 3 months of age with progressive motor impairment appearing at 6 months, progressive neurodegeneration starting at 9 months, and hypokinesis at 12 months and neuronal intranuclear inclusions at 18 months | Median survival 60-67 weeks of age |