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

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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

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  1. UMR 7102-Neurobiologie des Processus Adaptatifs (NPA): CNRS, Universit’e Pierre et Marie Curie-Paris6, UMR 7102-NPA, 9, quai StBernard, F75005, Paris, France

    Fatiha Boukhtouche, Mohamed Doulazmi, Florence Frederic, Isabelle Dusart, Bernard Brugg & Jean Mariani

  2. AP-HP, Hôpital Charles Foix, UEF, Ivry sur Seine, F94200, France

    Jean Mariani

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  1. Fatiha Boukhtouche
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Boukhtouche, F., Doulazmi, M., Frederic, F. et al. Rorα, a pivotal nuclear receptor for Purkinje neuron survival and differentiation: From development to ageing. Cerebellum 5, 97–104 (2006). https://doi.org/10.1080/14734220600750184

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