R6/2 Mouse

Charles River conducts contract studies in transgenic R6/2 mice to test the efficacy of novel therapeutics for treating Huntington’s disease (HD). All studies can be customized according to client needs.

The transgenic R6/2 mouse [B6CBA-Tg(HDexon1)62Gpb/1J] is the most commonly used animal model of Huntington’s disease. The R6/2 mouse contains N-terminally truncated mutant Htt (mHtt) with CAG repeat expansion (~125 repeats) within the huntingtin gene exon 1. This model develops HD-like symptoms, including decreased body weight as well as motor and cognitive deficits, starting as early as 6-8 weeks of age. Survival is followed until 25 weeks of age.

Study Components
Additional Endpoints

Body weight
Motor deficits
- Open field test
- Rotarod test
- Grip strength test
- Rear climbing test
Cognitive deficits
- Two-choice swim test
- Contextual fear conditioning
Neurological index
In vivo brain imaging
- MRI for brain volumetry (whole brain, cortex, striatum)
- MRS for striatal metabolites

Several additional endpoints are available for evaluating the lead compound method of action, such as IHC for mutated Huntingtin protein, inflammatory markers, iron accumulation and dopaminergic neurotransmission for intensity analysis or stereological counting, and qPCR assays for various transcripts.