The Cerebellum

, 5:97

Rorα, a pivotal nuclear receptor for Purkinje neuron survival and differentiation: From development to ageing

  • Fatiha Boukhtouche
  • Mohamed Doulazmi
  • Florence Frederic
  • Isabelle Dusart
  • Bernard Brugg
  • Jean Mariani
Original Article Scientific Papers

DOI: 10.1080/14734220600750184

Cite this article as:
Boukhtouche, F., Doulazmi, M., Frederic, F. et al. Cerebellum (2006) 5: 97. doi:10.1080/14734220600750184

Abstract

RORα (Retinoid-relatedOrphanReceptor) is a transcription factor belonging to the superfamily of nuclear receptors. The spontaneousstaggerer (sg) mutation, which consists of a deletion in theRora gene, has been shown to cause the loss of function of the RORα protein. The total loss of RORα expression leads to cerebellar developmental defects, particularly to a dramatic decreased survival of Purkinje cells and an early block in the differentiation process. This review focuses on recent studies which position RORα as a pivotal factor controlling Purkinje cell survival and differentiation, from development to ageing

Key words

RORαPurkinje cellsstaggerer mutation

Copyright information

© Springer 2006

Authors and Affiliations

  • Fatiha Boukhtouche
    • 1
  • Mohamed Doulazmi
    • 1
  • Florence Frederic
    • 1
  • Isabelle Dusart
    • 1
  • Bernard Brugg
    • 1
  • Jean Mariani
    • 1
    • 2
  1. 1.UMR 7102-Neurobiologie des Processus Adaptatifs (NPA): CNRSUniversit’e Pierre et Marie Curie-Paris6ParisFrance
  2. 2.AP-HP, Hôpital Charles Foix, UEF, Ivry sur SeineFrance
  3. 3.UMR 7102, DVSN, Case 14, Universit’e Pierre et Marie CurieParisFrance