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Lurcher Mouse

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Essentials of Cerebellum and Cerebellar Disorders

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Abstract

Lurcher mutant mouse is a natural model of hereditary cerebellar degeneration which is caused by a mutation in the δ2 glutamate receptor encoding gene. Heterozygous Lurcher mice suffer from virtually complete loss of Purkinje cells and the degeneration of cerebellar interneurons, deep cerebellar nuclei, and inferior olive neurons. Progressive cerebellar degeneration in Lurcher mice affects motor and cognitive functions, as well as emotional processing and behavior.

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References

  • Araki K, Meguro H, Kushiya E, Takayama C, Inoue Y, Mishina M (1993) Selective expression of the glutamate receptor channel delta 2 subunit in cerebellar Purkinje cells. Biochem Biophys Res Commun 197:1267–1276

    Article  CAS  PubMed  Google Scholar 

  • Belzung C, Chapillon P, Lalonde R (2001) The effects of the lurcher mutation on object localization, T-maze discrimination, and radial arm maze tasks. Behav Genet 31:151–155

    Article  CAS  PubMed  Google Scholar 

  • Caddy KW, Biscoe TJ (1979) Structural and quantitative studies on the normal C3H and Lurcher mutant mouse. Philos Trans R Soc Lond Ser B Biol Sci 287:167–201

    CAS  Google Scholar 

  • Cairns J, Swanson D, Yeung J, Sinova A, Chan R, Potluri P, Dickson P, Mittleman G, Goldowitz D (2017) Abnormalities in the structure and function of cerebellar neurons and neuroglia in the Lc/+ chimeric mouse model of variable developmental purkinje cell loss. Cerebellum 16:40–54

    Article  CAS  PubMed  Google Scholar 

  • Caston J, Chianale C, Delhaye-Bouchaud N, Mariani J (1998) Role of the cerebellum in exploration behavior. Brain Res 808:232–237

    Article  CAS  PubMed  Google Scholar 

  • Cendelin J, Voller J, Vozeh F (2010) Ataxic gait analysis in a mouse model of the olivocerebellar degeneration. Behav Brain Res 210:8–15

    Article  PubMed  Google Scholar 

  • Cendelin J, Tuma J, Korelusova I, Vozeh F (2014) The effect of genetic background on behavioral manifestation of Grid2(Lc) mutation. Behav Brain Res 271:218–227

    Article  CAS  PubMed  Google Scholar 

  • Coutelier M, Burglen L, Mundwiller E, Abada-Bendib M, Rodriguez D, Chantot-Bastaraud S, Rougeot C, Cournelle MA, Milh M, Toutain A, Bacq D, Meyer V, Afenjar A, Deleuze JF, Brice A, Heron D, Stevanin G, Durr A (2015) GRID2 mutations span from congenital to mild adult-onset cerebellar ataxia. Neurology 84:1751–1759

    Article  CAS  PubMed  Google Scholar 

  • De Jager PL, Zuo J, Cook SA, Heintz N (1997) A new allele of the lurcher gene, lurcherJ. Mamm Genome 8:647–650

    Article  PubMed  Google Scholar 

  • Duffin CA, McFarland R, Sarna JR, Vogel MW, Armstrong CL (2010) Heat shock protein 25 expression and preferential Purkinje cell survival in the lurcher mutant mouse cerebellum. J Comp Neurol 518:1892–1907

    Article  CAS  PubMed  Google Scholar 

  • Dumesnil-Bousez N, Sotelo C (1992) Early development of the Lurcher cerebellum: Purkinje cell alterations and impairment of synaptogenesis. J Neurocytol 21:506–529

    Article  CAS  PubMed  Google Scholar 

  • Fortier PA, Smith AM, Rossignol S (1987) Locomotor deficits in the mutant mouse, Lurcher. Exp Brain Res 66:271–286

    Article  CAS  PubMed  Google Scholar 

  • Frederic F, Chautard T, Brochard R, Chianale C, Wollman E, Oliver C, Delhaye-Bouchaud N, Mariani J (1997) Enhanced endocrine response to novel environment stress and endotoxin in Lurcher mutant mice. Neuroendocrinology 66:341–347

    Article  CAS  PubMed  Google Scholar 

  • Heckroth JA (1994) Quantitative morphological analysis of the cerebellar nuclei in normal and lurcher mutant mice. I. Morphology and cell number. J Comp Neurol 343:173–182

    Article  CAS  PubMed  Google Scholar 

  • Hilber P, Jouen F, Delhaye-Bouchaud N, Mariani J, Caston J (1998) Differential roles of cerebellar cortex and deep cerebellar nuclei in learning and retention of a spatial task: studies in intact and cerebellectomized lurcher mutant mice. Behav Genet 28:299–308

    Article  CAS  PubMed  Google Scholar 

  • Hilber P, Lalonde R, Caston J (1999) An unsteady platform test for measuring static equilibrium in mice. J Neurosci Methods 88:201–205

    Article  CAS  PubMed  Google Scholar 

  • Hilber P, Caston J (2001) Motor skills and motor learning in Lurcher mutant mice during aging. Neuroscience 102:615–623

    Article  CAS  PubMed  Google Scholar 

  • Hilber P, Lorivel T, Delarue C, Caston J (2004) Stress and anxious-related behaviors in Lurcher mutant mice. Brain Res 1003:108–112

    Article  CAS  PubMed  Google Scholar 

  • Cheng SS, Heintz N (1997) Massive loss of mid- and hindbrain neurons during embryonic development of homozygous lurcher mice. J Neurosci 17:2400–2407

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Jindrová A, Tuma J, Sládek V (2016) Impact of non-invasively induced motor deficits on tibial cortical properties in mutant lurcher mice. PLoS One 11:e0158877

    Article  PubMed  PubMed Central  Google Scholar 

  • Kolinko Y, Cendelin J, Kralickova M, Tonar Z (2016) Smaller absolute quantities but greater relative densities of microvessels are associated with cerebellar degeneration in lurcher mice. Front Neuroanat 10:35

    Article  PubMed  PubMed Central  Google Scholar 

  • Lalonde R, Lamarre Y, Smith AM (1988) Does the mutant mouse lurcher have deficits in spatially oriented behaviours? Brain Res 455:24–30

    Article  CAS  PubMed  Google Scholar 

  • Lalonde R, Thifault S (1994) Absence of an association between motor coordination and spatial orientation in lurcher mutant mice. Behav Genet 24:497–501

    Article  CAS  PubMed  Google Scholar 

  • Lalonde R (1998) Immobility responses in Lurcher mutant mice. Behav Genet 28:309–314

    Article  CAS  PubMed  Google Scholar 

  • Le Marec N, Caston J, Lalonde R (1997) Impaired motor skills on static and mobile beams in lurcher mutant mice. Exp Brain Res 116:131–138

    Article  PubMed  Google Scholar 

  • Lopez-Ramos JC, Houdek Z, Cendelin J, Vozeh F, Delgado-Garcia JM (2018) Timing correlations between cerebellar interpositus neuronal firing and classically conditioned eyelid responses in wild-type and Lurcher mice. Sci Rep 8:10697

    Article  PubMed  PubMed Central  Google Scholar 

  • Lorivel T, Roy V, Hilber P (2014) Fear-related behaviors in Lurcher mutant mice exposed to a predator. Genes Brain Behav 13:794–801

    Article  CAS  PubMed  Google Scholar 

  • Lorivel T, Cendelin J, Hilber P (2021) Familiarization effects on the behavioral disinhibition of the cerebellar Lurcher mutant mice: use of the innovative Dual Maze. Behav Brain Res 398:112972

    Article  CAS  PubMed  Google Scholar 

  • Nishiyama J, Yuzaki M (2010) Excitotoxicity and autophagy: lurcher may not be a model of “autophagic cell death”. Autophagy 6:568–570

    Article  PubMed  Google Scholar 

  • Norman DJ, Feng L, Cheng SS, Gubbay J, Chan E, Heintz N (1995) The lurcher gene induces apoptotic death in cerebellar Purkinje cells. Development 121:1183–1193

    Article  CAS  PubMed  Google Scholar 

  • Phillips RJS (1960) “Lurcher”, a new gene in linkage group XI of the house mouse. J Genet 57:35–42

    Article  Google Scholar 

  • Porras-Garcia E, Cendelin J, Dominguez-del-Toro E, Vozeh F, Delgado-Garcia JM (2005) Purkinje cell loss affects differentially the execution, acquisition and prepulse inhibition of skeletal and facial motor responses in Lurcher mice. Eur J Neurosci 21:979–988

    Article  PubMed  Google Scholar 

  • Purkartová Z, Vožeh F (2013) Cerebellar degeneration in Lurcher mice under confocal laser scanning microscope. Microsc Res Tech 76:545–551

    Article  PubMed  Google Scholar 

  • Rogers TD, Dickson PE, McKimm E, Heck DH, Goldowitz D, Blaha CD, Mittleman G (2013) Reorganization of circuits underlying cerebellar modulation of prefrontal cortical dopamine in mouse models of autism spectrum disorder. Cerebellum 12:547–556

    Article  CAS  PubMed  Google Scholar 

  • Selimi F, Doughty M, Delhaye-Bouchaud N, Mariani J (2000) Target-related and intrinsic neuronal death in Lurcher mutant mice are both mediated by caspase-3 activation. J Neurosci 20:992–1000

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Sultan F, Konig T, Mock M, Thier P (2002) Quantitative organization of neurotransmitters in the deep cerebellar nuclei of the Lurcher mutant. J Comp Neurol 452:311–323

    Article  CAS  PubMed  Google Scholar 

  • Thullier F, Lalonde R, Cousin X, Lestienne F (1997) Neurobehavioral evaluation of lurcher mutant mice during ontogeny. Brain Res Dev Brain Res 100:22–28

    Article  CAS  PubMed  Google Scholar 

  • Tuma J, Kolinko Y, Vozeh F, Cendelin J (2015) Mutation-related differences in exploratory, spatial, and depressive-like behavior in pcd and Lurcher cerebellar mutant mice. Front Behav Neurosci 9:116

    Article  PubMed  PubMed Central  Google Scholar 

  • Tuma J, Kolinko Y, Jelinkova D, Hilber P, Cendelin J (2017) Impaired spatial performance in cerebellar-deficient Lurcher mice is not associated with their abnormal stress response. Neurobiol Learn Mem 140:62–70

    Article  PubMed  Google Scholar 

  • Van Alphen AM, Schepers T, Luo C, De Zeeuw CI (2002) Motor performance and motor learning in Lurcher mice. Ann N Y Acad Sci 978:413–424

    Article  PubMed  Google Scholar 

  • Vernet-der Garabedian B, Lemaigre-Dubreuil Y, Delhaye-Bouchaud N, Mariani J (1998) Abnormal IL-1beta cytokine expression in the cerebellum of the ataxic mutant mice staggerer and lurcher. Brain Res Mol Brain Res 62:224–227

    Article  CAS  PubMed  Google Scholar 

  • Vernet-der Garabedian B, Derer P, Bailly Y, Mariani J (2013) Innate immunity in the Grid2Lc/+ mouse model of cerebellar neurodegeneration: glial CD95/CD95L plays a non-apoptotic role in persistent neuron loss-associated inflammatory reactions in the cerebellum. J Neuroinflammation 10:65

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Vogel MW, Sunter K, Herrup K (1989) Numerical matching between granule and Purkinje cells in lurcher chimeric mice: a hypothesis for the trophic rescue of granule cells from target-related cell death. J Neurosci 9:3454–3462

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Wang QJ, Ding Y, Kohtz DS, Mizushima N, Cristea IM, Rout MP, Chait BT, Zhong Y, Heintz N, Yue Z (2006) Induction of autophagy in axonal dystrophy and degeneration. J Neurosci 26:8057–8068

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Wetts R, Herrup K (1982a) Interaction of granule, Purkinje and inferior olivary neurons in lurcher chimaeric mice. I. Qualitative studies. J Embryol Exp Morpholog 68:87–98

    CAS  Google Scholar 

  • Wetts R, Herrup K (1982b) Interaction of granule, Purkinje and inferior olivary neurons in lurcher chimeric mice. II. Granule cell death. Brain Res 250:358–362

    Article  CAS  PubMed  Google Scholar 

  • Yue Z, Horton A, Bravin M, DeJager PL, Selimi F, Heintz N (2002) A novel protein complex linking the delta 2 glutamate receptor and autophagy: implications for neurodegeneration in lurcher mice. Neuron 35:921–933

    Article  CAS  PubMed  Google Scholar 

  • Zanjani HS, Lohof AM, McFarland R, Vogel MW, Mariani J (2013) Enhanced survival of wild-type and Lurcher Purkinje cells in vitro following inhibition of conventional PKCs or stress-activated MAP kinase pathways. Cerebellum 12:377–389

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Zanjani SH, Selimi F, Vogel MW, Haeberle AM, Boeuf J, Mariani J, Bailly YJ (2006) Survival of interneurons and parallel fiber synapses in a cerebellar cortex deprived of Purkinje cells: studies in the double mutant mouse Grid2Lc/+;Bax(−/−). J Comp Neurol 497:622–635

    Article  PubMed  Google Scholar 

  • Zuo J, De Jager PL, Takahashi KA, Jiang W, Linden DJ, Heintz N (1997) Neurodegeneration in Lurcher mice caused by mutation in delta2 glutamate receptor gene. Nature 388:769–773

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Pathophysiology, Faculty of Medicine in Pilsen, Charles University, Plzen, Czech Republic

    Jan Cendelin, Jan Tuma & Zdenka Purkartova

  2. Laboratory of Neurodegenerative Disorders, Biomedical Center, Faculty of Medicine in Pilsen, Charles University, Plzen, Czech Republic

    Jan Cendelin

Authors
  1. Jan Cendelin
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  2. Jan Tuma
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  3. Zdenka Purkartova
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Corresponding author

Correspondence to Jan Cendelin .

Editor information

Editors and Affiliations

  1. Neuroscience Department, The Scripps Research Institute, La Jolla, CA, USA

    Donna L. Gruol

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

    Noriyuki Koibuchi

  3. Service de Neurologie, CHU-Charleroi, Charleroi, Belgium

    Mario Manto

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

    Marco Molinari

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

    Jeremy D. Schmahmann

  6. Department of Physiology, Zhejiang University School of Medicine, Hangzho, China

    Ying Shen

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Cendelin, J., Tuma, J., Purkartova, Z. (2023). Lurcher Mouse. In: Gruol, D.L., Koibuchi, N., Manto, M., Molinari, M., Schmahmann, J.D., Shen, Y. (eds) Essentials of Cerebellum and Cerebellar Disorders. Springer, Cham. https://doi.org/10.1007/978-3-031-15070-8_63

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