Huntington's Disease

The severe disease bearing the name of American physician George Huntington has been known under various other names, including Chorea major, or St. Vitus Dance, for a long time. Modern molecular genetics revealed that Huntington’s disease is caused by a mutation in the gene coding for the protein Huntingtin. The altered protein cannot be degraded properly and aggregates predominantly in parts of the basal ganglia, leading to neuronal cell death.

A sufficient treatment for the disease has not been found yet. With an average duration between 15 and 20 years, Huntington’s disease is lethal. It is characterized by cognitive decline, locomotion deficits and psychiatric problems including anxiety.

As for other diseases, animal models, including knock-out and transgenic rodents have been developed which show some resemblance to human symptoms and allow the study of underlying mechanisms and possible treatment strategies. These animal models have been characterized both for behavioral traits and motor deficits. In this respect, testing paradigms such as the RotaRod, balance beam ladder running like in the MotoRater or complex activity wheels which represent one module of the PhenoMaster System, have been used.

For the investigation of anxiety-like behavior, TSE Systems offers a state-of-the-art video-tracking system to measure animal behavior in an open field, the elevated plus or zero maze or the classical light-dark test, which can be applied as fully automated module of the Multi Conditioning System. With this Multi Conditioning System it is also possible to investigate learning and memory deficits, which are a characteristic of many animal models of Huntington’s disease. Fear conditioning, active and passive avoidance would be modules of interest. Since the disease develops over time, longitudinal studies of learning and memory within the home cage may have to be performed. TSE Systems here offer state-of-the-art automated solutions such as PhenoMaster and IntelliCage by NewBehavior, which can be supplemented by classical behavioral paradigms like the Morris water maze.