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PMID: 22072660 Published · ppublish English Journal Article Research Support, N.I.H., Extramural Review

Local RNA translation at the synapse and in disease.

The Journal of neuroscience : the official journal of the Society for Neuroscience ·Vol. 31 ·No. 45 ·2011-11-09 ·Pages 16086-93

Liu-Yesucevitz L, Bassell GJ, Gitler AD, Hart AC, Klann E, Richter JD, Warren ST, Wolozin B

Abstract

Local regulation of protein synthesis in neurons has emerged as a leading research focus because of its importance in synaptic plasticity and neurological diseases. The complexity of neuronal subcellular domains and their distance from the soma demand local spatial and temporal control of protein synthesis. Synthesis of many synaptic proteins, such as GluR and PSD-95, is under local control. mRNA binding proteins (RBPs), such as FMRP, function as key regulators of local RNA translation, and the mTORC1 pathway acts as a primary signaling cascade for regulation of these proteins. Much of the regulation occurs through structures termed RNA granules, which are based on reversible aggregation of the RBPs, some of which have aggregation prone domains with sequence features similar to yeast prion proteins. Mutations in many of these RBPs are associated with neurological diseases, including FMRP in fragile X syndrome; TDP-43, FUS (fused in sarcoma), angiogenin, and ataxin-2 in amyotrophic lateral sclerosis; ataxin-2 in spinocerebellar ataxia; and SMN (survival of motor neuron protein) in spinal muscular atrophy.

MeSH Terms
Animals Humans Nerve Tissue Proteins/genetics,metabolism Nervous System Diseases/genetics,metabolism,pathology Neurons/metabolism Protein Biosynthesis/physiology RNA/genetics,metabolism RNA-Binding Proteins/genetics,metabolism Signal Transduction/genetics Synapses/genetics,metabolism
Chemicals
Nerve Tissue Proteins RNA-Binding Proteins RNA
Authors & Affiliations
8 authors, click to expand affiliations / ORCID
Liu-Yesucevitz Liqun
Department of Pharmacology, Boston University School of Medicine, Boston, Massachusetts 02118, USA.
Bassell Gary J
Gitler Aaron D
Hart Anne C
Klann Eric
Richter Joel D
Warren Stephen T
Wolozin Benjamin
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Article Info
Journal
The Journal of neuroscience : the official journal of the Society for Neuroscience
Abbr.
J Neurosci
ISSN
1529-2401
DOI
10.1523/JNEUROSCI.4105-11.2011
Published
2011-11-09
Pages
16086-93
Language
English
Region
United States
NLM ID
8102140
PMCID
PMC3241995
Subset
IM
Grants
NIEHS NIH HHS · ES15567 · United States
NINDS NIH HHS · R01 NS060872-02 · United States
NINDS NIH HHS · R21 NS066108 · United States
NINDS NIH HHS · R21 NS073679 · United States
NINDS NIH HHS · R01 NS060872-03 · United States
NINDS NIH HHS · R21 NS073679-01A1 · United States
NINDS NIH HHS · R21 NS073679-02 · United States
NINDS NIH HHS · R01 NS060872-02S1 · United States
NINDS NIH HHS · R01 NS060872 · United States
NINDS NIH HHS · R01 NS060872-04 · United States
NINDS NIH HHS · NS066108 · United States
NIEHS NIH HHS · R01 ES020395 · United States
NINDS NIH HHS · R21 NS066108-01A1 · United States
NINDS NIH HHS · R21 NS066108-02 · United States
NIEHS NIH HHS · R01 ES015567 · United States
NINDS NIH HHS · NS060872 · United States
External Links
PubMed source DOI: 10.1523/JNEUROSCI.4105-11.2011 PMC Full Text View journal details
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