Symptomatic animal models for dystonia

• Type of Material: Article
• Authors: Bethany K. Wilson, Emory University; Ellen Hess, Emory University
• Language: English
• Date: 2013-06-15
• Publisher: Wiley
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2013 Movement Disorder Society
• Final Published Version (URL): http://onlinelibrary.wiley.com/doi/10.1002/mds.25526/abstract
• Title of Journal or Parent Work: Movement Disorders
• ISSN: 0885-3185
• Volume: 28
• Issue: 7
• Start Page: 982
• End Page: 989
• Grant/Funding Information: This work was supported by the Bachmann-Strauss Dystonia and Parkinson Foundation and United States National Institutes of Health R01 NS33592 and T32 GM8605.; BKW was supported by a training grant from the National Institutes of Health.; Grants from the National Institutes of Health and the Bachmann-Strauss Dystonia & Parkinson Foundation and research contracts from Merck Pharmaceuticals and Addex Pharmaceuticals were awarded to EJH.
• Keywords: cerebellum; basal ganglia; mouse model; primate model
• Research Categories: Biology, Neuroscience; Health Sciences, General

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 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.