Huntington’s disease (HD) is an autosomal-dominantly inherited, fatal, neurodegenerative disorder. Patients present with motor dysfunction, psychiatric disturbances, cognitive impairments, and metabolic abnormalities. The sole cause of developing HD is the expansion of an unstable repeat of CAG base triplets in the coding region of the Huntingtin gene, HTT. CAG repeat lengths of up to 34 are considered to be physiological, while more than 35 CAG repeats lead most likely to the development of HD. The age of disease onset correlates inversely with CAG repeat length and starts at the age of 40–50 years.
Scantox currently offers several Huntington’s Disease mouse models featuring different properties with regard to Htt expression patterns, neuroinflammation, behavioral deficits, age at onset and progression of pathology. These animals focus on different pathological readouts and constitute suitable models to study the influence of drugs on Htt-related brain pathology and behavior.
Scantox offers custom tailored study design for these models, and we are flexible to accommodate to your special interests. We are also happy to advice you and propose study designs. Scantox maintains its own colonies directly in our research facility. Non-transgenic littermates are available as control animals needed for proper study design.
We would be happy to test your compounds in these mouse models! Readouts depend on model but the most common are:
- Survival
- Body weight
- HTT aggregates
- Motor deficits
- Learning and relearning deficits
- Anxiety
- Striatal alterations
- Neuroinflammation
Looking for something else? Please contact us!
You might be also interested in these related models:
As with all other in vivo models we are also ready to provide samples (brain tissue, CSF etc.) from these animals for analyses in your laboratory.