Chorea Huntington: Animal models direct the way to a better pathophysiological understanding and new therapeutical approaches

Huntington's disease (HD) is member of a growing family of neurodegenerativ e diseases which are caused by a CAG-Triplet expansion in the coding region of their respective genes. The results of the research of the last years i s very suggestive of a common pathomechanisms of all these diseases even th ough their clinical appearance may be quite different. The development of n ew animal models by transferring the human gene defect into the mouse genom e has led to the finding of so-called intranuclear inclusion bodies. This n ew observation allowed to come closer to solving the problem how this genet ic defect causes neurodegeneration. Recent studies on transgenic HD mice co uld also demonstrate a possible connection between the genetic defect and g lutamate exitotoxicity in the neurodegenerative process of HD which had bee n emphasized by earlier animal models of the disease. Transgenic animal mod els of HD will have an important impact on the understanding of the disease mechanisms and may contribute to a faster development and testing of new t herapeutic approaches.