The use of the R6 transgenic mouse models of Huntington's disease in attempts to develop novel therapeutic strategies.

Research output: Contribution to journalArticle


Huntington's disease (HD) is a genetic neurodegenerative disorder. Since identification of the disease-causing gene in 1993, a number of genetically modified animal models of HD have been generated. The first transgenic mouse models, R6/1 and R6/2 lines, were established 8 years ago. The R6/2 mice have been the best characterized and the most widely used model to study pathogenesis of HD and therapeutic interventions. In the present review, we especially focus on the characteristics of R6 transgenic mouse models and, in greater detail, describe the different therapeutic strategies that have been tested in these mice. We also, at the end, critically assess the relevance of the HD mouse models compared with the human disease and discuss how they can be best used in the future.


Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Neurosciences


  • Huntington Disease: psychology, Huntington Disease: therapy, Mice, Huntington Disease: pathology, Behavior, Animal, Humans, Huntington Disease: diet therapy, Huntington Disease: genetics, Animals, Anti-Inflammatory Agents: therapeutic use, Apoptosis: drug effects, Brain: pathology, Disease Models, Transgenic: physiology, Protease Inhibitors: therapeutic use, Protein Folding, Research Support, Non-U.S. Gov't, Tissue Therapy, Transglutaminases: antagonists & inhibitors
Original languageEnglish
Pages (from-to)447-64
Issue number3
Publication statusPublished - 2005
Publication categoryResearch

Bibliographic note

The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Neuronal Survival (013212041)