Huntington's Disease Model
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Transgenic R6/2 mice [B6CBA-Tg(HDexon1)62Gpb/1J] with CAG repeat expansion (~125 repeats) in huntington gene exon 1 is the most commonly used model to assess compound efficacy for alleviating the deficits of the phenotype in short and longitudinal studies. Charles River’s Behavioral and Cognitive Test Panel can be supplemented with tests modeling psychiatric symptoms of the disease. In vivo profiling of striatal metabolites with Magnetic Resonance Spectroscopy provides data for metabolic changes by compound, and Magnetic Resonance Imaging of brain volumetry indications for effects on atrophy. All studies can be customized according to client needs.
Example Study Paradigm of Huntington's Disease Model

Click here to see a diagram outlining an example study paradigm using our Huntington's disease model.
Behavioral and Cognitive Tests for Huntington’s Disease Motor Deficits
- Open Field Test
- Distance traveled
- Rearing frequency
- Velocity
- Multiple other variables
- Rotarod
- Grip Strength
- Rear Climbing
- Rearing behavior
- Climbing behavior
Cognitive Deficits
Charles River offers a variety of brain imaging modalities. In vivo Magnetic Resonance Spectroscopy for Metabolites:
- Gamma-aminobutyric acid (GABA)
- Glutamine (GLN)
- Glutamate (GLU)
- Myo-inositol (INS)
- N-acetylaspartate (NAA)
- Taurine (TAU)
- Choline (CHO)
- N-acetylaspartate + N-acetylaspartylglutamate (NAA+NAAG)
- Creatine + phosphocreatine (CR+PCR)
- Glutamate + glutamine (GLU+GLN)

R6/2 MR Spectroscopy Compound X
In vivo and ex vivo Magnetic Resonance Imaging for Brain Volumetry
- Whole Brain
- Striatum
- Cortex

For more information about our Huntington’s disease research studies with transgenic R6/2 mice, please contact us at 1.877.CRIVER.1 or askcharlesriver@crl.com.
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Huntington’s disease, Transgenic R6/2 mice, CAG repeat, huntington gene exon 1, striatal metabolites, Open Field Test, Rotarod, Grip Strength, Two-choice swim test, Contextual Fear Conditioning, Gamma-aminobutyric acid, GABA, Glutamine, GLN, Glutamate, GLU, Myo-inositol, INS, N-acetylaspartate, NAA, Taurine, TAU, Choline, CHO, N-acetylaspartate, N-acetylaspartylglutamate, NAAG, Creatine, phosphocreatine. CR+PCR
Huntington’s Disease Research Studies with Transgenic R6/2 Mice | Charles River
Transgenic R6/2 mice with CAG repeat expansion (~125 repeats) in huntington gene exon 1 is the most commonly used model to assess compound efficacy for alleviating the deficits of Huntington’s disease.