RORalpha, a pivotal nuclear receptor for Purkinje neuron survival and differentiation: from development to ageing.
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.
Boukhtouche
Fatiha
F
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.
Doulazmi
Mohamed
M
Frederic
Florence
F
Dusart
Isabelle
I
Brugg
Bernard
B
Mariani
Jean
J
D000818:Animals; D002454:Cell Differentiation / Q000235:genetics; D002470:Cell Survival / Q000235:genetics; D016922:Cellular Senescence / Q000235:genetics; D002525:Cerebellar Cortex / Q000166:cytology / Q000196:embryology* / Q000254:growth & development*; D019610:Cytoprotection / Q000235:genetics; D018507:Gene Expression Regulation, Developmental / Q000235:genetics; D051379:Mice; D009154:Mutation / Q000235:genetics; D057094:Nuclear Receptor Subfamily 1, Group F, Member 1; D011689:Purkinje Cells / Q000166:cytology / Q000378:metabolism*; D018160:Receptors, Cytoplasmic and Nuclear / Q000235:genetics* / Q000378:metabolism*; D015534:Trans-Activators / Q000235:genetics* / Q000378:metabolism*
D057094:Nuclear Receptor Subfamily 1, Group F, Member 1; D018160:Receptors, Cytoplasmic and Nuclear; C539739:Rora protein, mouse; D015534:Trans-Activators