| Literature DB >> 16818384 |
Fatiha Boukhtouche1, Mohamed Doulazmi, Florence Frederic, Isabelle Dusart, Bernard Brugg, Jean Mariani.
Abstract
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.Entities:
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Year: 2006 PMID: 16818384 DOI: 10.1080/14734220600750184
Source DB: PubMed Journal: Cerebellum ISSN: 1473-4222 Impact factor: 3.648