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Lurcher Mouse

  • Reference work entry
Handbook of the Cerebellum and Cerebellar Disorders

Abstract

Lurcher mutant mice represent one of the frequently used mouse models of the olivocerebellar degeneration. It is caused by a mutation in the δ2 glutamate receptor subunit encoding gene. The gain of function mutation changes the receptor into a leaky membrane channel leading to chronic depolarization of the cells expressing the receptor. Heterozygous Lurcher mice suffer from virtually complete postnatal loss of cerebellar Purkinje cells and reduction of granule, stellate and basket cells, and inferior olive neurons and relatively mild changes in the deep cerebellar nuclei. The death of Purkinje cells is a primary effect of the mutation and it shows features of apoptosis, autophagy, and necrosis. Extinction of the granule, stellate and basket cells, and inferior olive neurons is a target-related cell death. Lurcher mice display neurochemical and metabolic changes, abnormalities in the neurotransmitter and receptor systems, endocrine and immune abnormalities, and multiple behavioral deficits.

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Authors and Affiliations

  1. Department of Pathophysiology, Faculty of Medicine in Pilsen, Charles University, Lidicka 1, 301 66, Pilsen, Czech Republic

    Dr. Jan Cendelín (Faculty of Medicine in Pilsen) & Prof. František Vožeh

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  1. Dr. Jan Cendelín
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  2. Prof. František Vožeh
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Correspondence to Jan Cendelín .

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Editors and Affiliations

  1. Unité d'Etude du Mouvement (UEM), FNRS, Neurologie ULB Erasme, Bruxelles, Belgium

    Mario Manto

  2. Ataxia Unit, Cognitive and Behavioral Neurology Unit, Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Jeremy D. Schmahmann

  3. Neuroscience Institute of the Cavalieri-Ottolenghi Foundation (NICO), University of Turin, Orbassano, Turin, Italy

    Ferdinando Rossi

  4. Molecular and Integrative Neuroscience Department (MIND), The Scripps Research Institute, California, CA, USA

    Donna L. Gruol

  5. Department of Integrative Physiolgy, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan

    Noriyuki Koibuchi

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Cendelín, J., Vožeh, F. (2013). Lurcher Mouse. In: Manto, M., Schmahmann, J.D., Rossi, F., Gruol, D.L., Koibuchi, N. (eds) Handbook of the Cerebellum and Cerebellar Disorders. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-1333-8_66

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