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Keywords:

  • dystonia;
  • pathogenesis;
  • animal models;
  • transgenic mice;
  • knockout mice

Abstract

A large number of different genetic and acquired disorders of the nervous system may be associated with dystonia. To elucidate its pathogenesis and to facilitate the discovery of potential novel treatments, there has been a growing interest in the development of animal models and particularly rodent models. Multiple animal models for dystonia have now been developed and partially characterized. The results obtained from studies of these models often lead in very different directions, in part because the different models target different aspects of a very heterogeneous disorder. A recent workshop addressed four main issues affecting those who conduct dystonia research with animal models, including the different ways in which dystonic disorders can be modeled in rodents, key features that constitute a useful model, methods used in the evaluation of these models, and recommendations for future research. This review summarizes the main outcomes of this conference. © 2005 Movement Disorder Society