Cerebellar neurodegeneration in the absence of microRNAs

Cerebellar neurodegeneration in the absence of microRNAs

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 diffe...

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Main Authors: Schaefer, Anne, O'Carroll, Dónal, Tan, Chan Lek, Hillman, Dean, Sugimori, Mutsuyuki, Llinas, Rodolfo, Greengard, Paul
Format: Online
Language:English
Published: The Rockefeller University Press 2007
Online Access:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2118654/
id pubmed-2118654
recordtype oai_dc
spelling pubmed-21186542008-01-09 Cerebellar neurodegeneration in the absence of microRNAs Schaefer, Anne O'Carroll, Dónal Tan, Chan Lek Hillman, Dean Sugimori, Mutsuyuki Llinas, Rodolfo Greengard, Paul Brief Definitive Reports 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. The Rockefeller University Press 2007-07-09 /pmc/articles/PMC2118654/ /pubmed/17606634 http://dx.doi.org/10.1084/jem.20070823 Text en Copyright © 2007, The Rockefeller University Press This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.rupress.org/terms). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 4.0 Unported license, as described at http://creativecommons.org/licenses/by-nc-sa/4.0/).
repository_type Open Access Journal
institution_category Foreign Institution
institution US National Center for Biotechnology Information
building NCBI PubMed
collection Online Access
language English
format Online
author Schaefer, Anne
O'Carroll, Dónal
Tan, Chan Lek
Hillman, Dean
Sugimori, Mutsuyuki
Llinas, Rodolfo
Greengard, Paul
spellingShingle Schaefer, Anne
O'Carroll, Dónal
Tan, Chan Lek
Hillman, Dean
Sugimori, Mutsuyuki
Llinas, Rodolfo
Greengard, Paul
Cerebellar neurodegeneration in the absence of microRNAs
author_facet Schaefer, Anne
O'Carroll, Dónal
Tan, Chan Lek
Hillman, Dean
Sugimori, Mutsuyuki
Llinas, Rodolfo
Greengard, Paul
author_sort Schaefer, Anne
title Cerebellar neurodegeneration in the absence of microRNAs
title_short Cerebellar neurodegeneration in the absence of microRNAs
title_full Cerebellar neurodegeneration in the absence of microRNAs
title_fullStr Cerebellar neurodegeneration in the absence of microRNAs
title_full_unstemmed Cerebellar neurodegeneration in the absence of microRNAs
title_sort cerebellar neurodegeneration in the absence of micrornas
description 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.
publisher The Rockefeller University Press
publishDate 2007
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2118654/
_version_ 1611414888224129024

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