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Review
. 2014 Jun 16:1:4.
doi: 10.1186/2053-8871-1-4. eCollection 2014.

From mice to men: lessons from mutant ataxic mice

Jan Cendelin  1
Affiliations
Review

From mice to men: lessons from mutant ataxic mice

Jan Cendelin. Cerebellum Ataxias. .

Abstract

Ataxic mutant mice can be used to represent models of cerebellar degenerative disorders. They serve for investigation of cerebellar function, pathogenesis of degenerative processes as well as of therapeutic approaches. Lurcher, Hot-foot, Purkinje cell degeneration, Nervous, Staggerer, Weaver, Reeler, and Scrambler mouse models and mouse models of SCA1, SCA2, SCA3, SCA6, SCA7, SCA23, DRPLA, Niemann-Pick disease and Friedreich ataxia are reviewed with special regard to cerebellar pathology, pathogenesis, functional changes and possible therapeutic influences, if any. Finally, benefits and limitations of mouse models are discussed.

Keywords: Ataxia; Cerebellum; Neurodegeneration.

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References

    1. Schmahmann JD, Sherman JC. Cerebellar cognitive affective syndrome. Int Rev Neurobiol. 1997;41:433–440. - PubMed
    1. Manto MU. The wide spectrum of spinocerebellar ataxias (SCAs) Cerebellum. 2005;4:2–6. - PubMed
    1. Manto M, Marmolino D. Animal models of human cerebellar ataxias: a cornerstone for the therapies of the twenty-first century. Cerebellum. 2009;8:137–154. - PubMed
    1. Phillips RJS. “Lurcher”, a new gene in linkage group XI of the house mouse. J Genet. 1960;57:35–42.
    1. Zuo J, De Jager PL, Takahasi KJ, Jiang W, Linden DJ, Heintz H. Neurodegeneration in Lurcher mice caused by mutation of δ2 glutamate receptor gene. Nature. 1997;388:769–773. - PubMed

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