From mice to men: lessons from mutant ataxic mice

Cerebellum & Ataxias
Jan Cendelin

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.

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Citations

Jan 2, 2020·International Journal of Molecular Sciences·Francesca PrestoriEgidio D'Angelo
Nov 20, 2016·The Cerebellum·František Vožeh
Jan 11, 2018·Journal of Neuroscience Research·Hermina NedelescuArwel T Pritchard
Nov 23, 2019·Proceedings of the National Academy of Sciences of the United States of America·Adi MinisHermann Steller
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Jul 29, 2020·ELife·Ana S MachadoMegan R Carey
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Mar 2, 2019·The Cerebellum·Robert Lalonde, Catherine Strazielle
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Aug 12, 2018·Nature Communications·Marija KojicBrandon J Wainwright
Jun 3, 2016·Frontiers in Cellular Neuroscience·Carolina CocitoLaura Lossi
Mar 2, 2018·The Journal of Clinical Investigation·Stephanie zur NeddenGabriele Baier-Bitterlich
Jan 30, 2021·Chinese Medicine·Michael Weng Lok PhangKah Hui Wong
Apr 6, 2021·Neurotoxicity Research·Samira EziReza Mastery Farahani
Aug 10, 2021·Current Protocols·Jacqueline M GarrickJudit Marsillach

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Methods Mentioned

BETA
transgenic
targeted mutations

Software Mentioned

Scrambler

Related Concepts

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Ataxias

Ataxia is a neurological condition characterized by lack of voluntary coordination of muscle movements including loss of coordination, balance, and speech. Discover the latest research on different types of ataxias here.