Chapter

Essentials of Cerebellum and Cerebellar Disorders

pp 463-468

Date:

Cellular and Animal Models of Cerebellar Disorders: Staggerer Mouse

  • N. MorelliniAffiliated withSorbonne Universités UPMC-Univ Paris 6 and CNRS, IBPS-B2A and DHU FAST, UMR8256 Biological Adaptation and Ageing Email author 
  • , A. M. LohofAffiliated withSorbonne Universités UPMC-Univ Paris 6 and CNRS, IBPS-B2A and DHU FAST, UMR8256 Biological Adaptation and Ageing
  • , R. M. SherrardAffiliated withSorbonne Universités UPMC-Univ Paris 6 and CNRS, IBPS-B2A and DHU FAST, UMR8256 Biological Adaptation and Ageing
  • , J. MarianiAffiliated withSorbonne Universités UPMC-Univ Paris 6 and CNRS, IBPS-B2A and DHU FAST, UMR8256 Biological Adaptation and AgeingInstitut de la Longévité, Hôpital Charles Foix

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Abstract

The staggerer mutant mouse carries a spontaneous mutation in the ligand-binding domain of the rora gene. RORα is expressed in many tissues and its loss leads to diverse abnormalities. In the cerebellum of staggerer mice, there is severe early degeneration of Purkinje cells and associated death of their afferent neurons (granule and olivary neurons). Thus staggerer mice have atrophic cerebella and associated severe ataxia. In contrast, although heterozygote staggerer mice develop apparently normally, there is premature Purkinje cell atrophy and death in adulthood. Given that recent links have been demonstrated between RORα and spinocerebellar ataxia and autism spectrum disorders, the staggerer mouse is a particularly interesting model for cerebellar pathologies.

Keywords

Purkinje Cell Neuroprotection Autism Spectrum Disorders Anti-Inflammatory Action Orphan Nuclear Receptor Spinocerebellar Ataxia