Boukhtouche Fatiha, Doulazmi Mohamed, Frederic Florence, Dusart Isabelle, Brugg Bernard, Mariani Jean
Université Pierre et Marie Curie-Paris6, UMR 7102 - Neurobiologie des Processus Adaptatifs (NPA): CNRS, UMR 7102-NPA, 9, quai St-Bernard, Paris, F-75005, France.
Cerebellum. 2006;5(2):97-104. doi: 10.1080/14734220600750184.
RORalpha (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 RORalpha protein. The total loss of RORalpha 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 RORalpha as a pivotal factor controlling Purkinje cell survival and differentiation, from development to ageing.
维甲酸相关孤儿受体α（RORα）是一种属于核受体超家族的转录因子。自发的蹒跚突变体（sg）由Rora基因的缺失组成，已被证明会导致RORα蛋白功能丧失。RORα表达的完全丧失会导致小脑发育缺陷，特别是浦肯野细胞的存活率显著降低以及分化过程的早期阻滞。这篇综述重点关注了近期的研究，这些研究将RORα定位为从发育到衰老过程中控制浦肯野细胞存活和分化的关键因子。
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