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The Tottering Mouse

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Essentials of Cerebellum and Cerebellar Disorders

Abstract

In the late 1950s, a novel spontaneous mutation was found in the same litter of mice while breeding by Green and Sidman at the Roscoe B. Jackson Memorial Laboratory in Maine. Mice harboring this mutation, which was found to be recessive, were termed tottering (tg/tg) mice, and exhibited a series of neurological abnormalities, including a paroxysmal motor disturbances (dyskinesia/dystonia), a wobbly ataxic gait, and absence seizures. Since the initial discovery of this mutation, numerous studies have investigated the underlying genetics and behavioral consequences in the tg/tg mouse, and this mouse has proven useful in our understanding of several episodic neurological disorders involving the cerebellum. Caused by a mutation in the Cacna1a gene that encodes the P/Q-type voltage-gated Ca channel, the tg/tg mouse is a model for the human disorder episodic ataxia type 2 (EA2), and has provided insights into the mechanisms of episodic cerebellar dysfunction.

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Authors and Affiliations

  1. Department of Neuroscience, University of Minnesota, 2001 6th St SE, Rm 421 LRB, Minneapolis, MN, 55455, USA

    Russell E. Carter & Timothy J. Ebner

Authors
  1. Russell E. Carter
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  2. Timothy J. Ebner
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Corresponding author

Correspondence to Timothy J. Ebner .

Editor information

Editors and Affiliations

  1. Molecular and Cellular Neuroscience Department, The Scripps Research Institute, La Jolla, California, USA

    Donna L. Gruol

  2. Department of Integrative Physiology, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan

    Noriyuki Koibuchi

  3. FNRS, ULB-Erasme, Bruxelles, Belgium

    Mario Manto

  4. Clinical Translational Research, Santa Lucia Foundation, Rome, Italy

    Marco Molinari

  5. Ataxia Unit, Cognitive Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA

    Jeremy D. Schmahmann

  6. Department of Neurobiology, Zhejiang University School of Medicine, Hangzhou, China

    Ying Shen

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© 2016 Springer International Publishing Switzerland

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Carter, R.E., Ebner, T.J. (2016). The Tottering Mouse. In: Gruol, D., Koibuchi, N., Manto, M., Molinari, M., Schmahmann, J., Shen, Y. (eds) Essentials of Cerebellum and Cerebellar Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-24551-5_59

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