Format

Send to

Choose Destination
See comment in PubMed Commons below
J Exp Med. 2007 Jul 9;204(7):1553-8. Epub 2007 Jul 2.

Cerebellar neurodegeneration in the absence of microRNAs.

Author information

1
Laboratory of Molecular and Cellular Neuroscience and 2Laboratory of Lymphocyte Signaling, The Rockefeller University, New York, NY 10021, USA.

Abstract

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.

PMID:
17606634
PMCID:
PMC2118654
DOI:
10.1084/jem.20070823
[Indexed for MEDLINE]
Free PMC Article
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for HighWire Icon for PubMed Central
    Loading ...
    Support Center