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.


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)81Dbo/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 huntington 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)81Dbo/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