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

被引:47
作者
Boukhtouche, Fatiha
Doulazmi, Mohamed
Frederic, Florence
Dusart, Isabelle
Brugg, Bernard
Mariani, Jean
机构
[1] Univ Paris 06, DVSN, CNRS, UMR Neurobiol Proc Adaptat 7102, F-75005 Paris, France
[2] UEF, Hop Charles Foix, AP HP, F-94200 Ivry, France
关键词
ROR alpha; Purkinje cells; staggerer mutation;
D O I
10.1080/14734220600750184
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
ROR alpha (Retinoid-related Orphan Receptor) is a transcription factor belonging to the superfamily of nuclear receptors. The spontaneous staggerer (sg) mutation, which consists of a deletion in the Rora gene, has been shown to cause the loss of function of the ROR alpha protein. The total loss of ROR alpha 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 ROR alpha as a pivotal factor controlling Purkinje cell survival and differentiation, from development to ageing.
引用
收藏
页码:97 / 104
页数:8
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