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

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.