Huntington's Disease Mouse Model Resource

Leverage the world’s largest collections of mouse models of Huntington’s disease and more than 80 years of neurobiology research experience.

See a complete list of JAX® Mice for Huntington's disease research

Comparison of Featured Huntington's Disease Models

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
B6CBA-Tg(HDexon1)62Gpb/1J (002810)	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
B6CBA-Tg(HDexon1)62Gpb/3J (006494)	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
B6.Cg-Tg(HDexon1)61Gpb/J (006471)	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
B6C3-Tg(HD82Gln)81Gschi/J (003627)	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
FVB-Tg(YAC128)53Hay/J (004938)	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

B6CBA-Tg(HDexon1)62Gpb/1J

(002810)

Common name: R6/2 transgenic
Genetic background: mixed, containing C57BL/6 and CBA
Expresses mutant human huntingtin (HTT) gene containing a polyglutamate-repeat (CAG) expansion of 160 +/- 5 (Mangiarini et al. 1996)
Progressive accumulation of neuronal intranuclear inclusions (NII) containing huntingtin and ubiquitin proteins
Develop choreiform-like and involuntary stereotypic movements, tremors, and epileptic seizures
Phenotype onset: 9-11 weeks of age

B6CBA-Tg(HDexon1)62Gpb/3J

(006494)

Common name: R6/2 substrain transgenic
Genetic background: mixed, containing C57BL/6 and CBA
Expresses mutant human huntingtin (HTT) gene containing a polyglutamate-repeat (CAG) expansion of 120 +/- 5 (this substrain with a shortened repeat expansion was developed from line 002810)
Progressive accumulation of neuronal intranuclear inclusions (NII) containing huntingtin and ubiquitin proteins
Develop choreiform-like and involuntary stereotypic movements, tremors, and epileptic seizures
Phenotype onset: 14-16 weeks of age

B6.Cg-Tg(HDexon1)61Gpb/J

(006471)

Common name: R6/1 transgenic
Genetic background: C57BL/6J (000664), incipient congenic (N5)
Expresses mutant human huntingtin (HTT) gene containing a polyglutamate-repeat (CAG) expansion of 115-150 in the 5’ end of the gene (Miller et al. 2005)
Progressive accumulation of neuronal intranuclear inclusions (NII) containing huntingtin and ubiquitin proteins
Develop choreiform-like and involuntary stereotypic movements, tremors, and epileptic seizures
Phenotype onset: 15-21 weeks of age

B6C3-Tg(HD82Gln)81Gschi/J

(003627)

Common name: HD-N171-82Q-81 transgenic
Genetic background: mixed, containing C57BL/6J (000664) and C3H/HeJ (000659)
Expresses N-terminally truncated (first 171 amino acids) human huntingtin (HTT) cDNA encoding 82 glutamines (Schilling et al. 1999)
Progressive accumulation of huntingtin positive nuclear inclusions in multiple neuron populations
Fail to gain weight, develop tremors, hypokinesis and lack of coordination after 2 months of age
Average life span: 5-6 months

FVB-Tg(YAC128)53Hay/J

(004938)

Common name: YAC128 transgenic
Genetic background: FVB/N, fully congenic
Expresses a yeast artificial chromosome (YAC) containing a full-length human huntingtin gene modified with a 128 CAG repeat expansion under the control of the endogenous human HDH promoter (Slow et al. 2003)
Nuclear huntingtin aggregate inclusions of striatal neurons at 18 months of age
Hyperkinesis beginning at 3 months of age with progressive motor impairment appearing at 6 months, followed by progressive neurodegeneration starting at 9 months, and hypokinesis at 12 months