Cerebellar neurodegeneration in the absence of microRNAs
| Autor: | Anne Schaefer, Chan Lek Tan, Mutsuyuki Sugimori, Dónal O'Carroll, Dean E. Hillman, Rodolfo R. Llinás, Paul Greengard |
|---|---|
| Rok vydání: | 2007 |
| Předmět: |
Cerebellum
Cellular differentiation Immunology Purkinje cell Apoptosis Biology Mice Purkinje Cells 03 medical and health sciences 0302 clinical medicine Gene expression microRNA In Situ Nick-End Labeling medicine Cerebellar Degeneration Animals Immunology and Allergy 030304 developmental biology 0303 health sciences Neurodegeneration Brief Definitive Report Cell Differentiation Cell Biology medicine.disease Immunohistochemistry Molecular biology Cell biology Disease Models Animal MicroRNAs medicine.anatomical_structure Nerve Degeneration biology.protein Brief Definitive Reports 030217 neurology & neurosurgery Dicer |
| Zdroj: | The Journal of Experimental Medicine |
| ISSN: | 1540-9538 0022-1007 |
| DOI: | 10.1084/jem.20070823 |
| Popis: | Genome-encoded microRNAs (miRNAs) are potent regulators of gene expression. The significance of miRNAs in various biological processes has been suggested by studies showing an important role of these small RNAs in regulation of cell differentiation. However, the role of miRNAs in regulation of differentiated cell physiology is not well established. Mature neurons express a large number of distinct miRNAs, but the role of miRNAs in postmitotic neurons has not been examined. Here, we provide evidence for an essential role of miRNAs in survival of differentiated neurons. We show that conditional Purkinje cell–specific ablation of the key miRNA-generating enzyme Dicer leads to Purkinje cell death. Deficiency in Dicer is associated with progressive loss of miRNAs, followed by cerebellar degeneration and development of ataxia. The progressive neurodegeneration in the absence of Dicer raises the possibility of an involvement of miRNAs in neurodegenerative disorders. |
| Databáze: | OpenAIRE |
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