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Animal models for dystonia

Authors

  • Bethany K. Wilson BS,

    1. Department of Pharmacology, Emory University School of Medicine, Atlanta, Georgia, USA
    2. Department of Neurology, Emory University School of Medicine, Atlanta, Georgia, USA
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  • Ellen J. Hess PhD

    Corresponding author
    1. Department of Neurology, Emory University School of Medicine, Atlanta, Georgia, USA
    • Department of Pharmacology, Emory University School of Medicine, Atlanta, Georgia, USA
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  • Relevant conflicts of interest/financial disclosures: Nothing to report.

  • Full financial disclosures and author roles may be found in the Acknowledgments section online.

Correspondence to: Dr. Ellen J. Hess, Departments of Pharmacology and Neurology, Emory University School of Medicine, 101 Woodruff Circle, WMB 6303, Atlanta, GA 30322, USA; ejhess@emory.edu

ABSTRACT

Symptomatic animal models have clinical features consistent with human disorders and are often used to identify the anatomical and physiological processes involved in the expression of symptoms and to experimentally demonstrate causality where it would be infeasible in the patient population. Rodent and primate models of dystonia have identified basal ganglia abnormalities, including alterations in striatal GABAergic (ie, transmitting or secreting γ-aminobutyric acid) and dopaminergic transmission. Symptomatic animal models have also established the critical role of the cerebellum in dystonia, particularly abnormal glutamate signaling and aberrant Purkinje cell activity. Further, experiments suggest that the basal ganglia and cerebellum are nodes in an integrated network that is dysfunctional in dystonia. The knowledge gained from experiments in symptomatic animal models may serve as the foundation for the development of novel therapeutic interventions to treat dystonia. © 2013 Movement Disorder Society

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