Abstract
De novo protein synthesis is necessary for long-lasting modifications in synaptic strength and dendritic spine dynamics that underlie cognition. Fragile X syndrome (FXS), characterized by intellectual disability and autistic behaviors, holds promise for revealing the molecular basis for these long-term changes in neuronal function. Loss of function of the fragile X mental retardation protein (FMRP) results in defects in synaptic plasticity and cognition in many models of the disease. FMRP is a polyribosome-associated RNA-binding protein that regulates the synthesis of a set of plasticity-reated proteins by stalling ribosomal translocation on target mRNAs. The recent identification of mRNA targets of FMRP and its upstream regulators, and the use of small molecules to stall ribosomes in the absence of FMRP, have the potential to be translated into new therapeutic avenues for the treatment of FXS.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 1530-1536 |
| Number of pages | 7 |
| Journal | Nature Neuroscience |
| Volume | 16 |
| Issue number | 11 |
| DOIs | |
| State | Published - Nov 2013 |
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ASJC Scopus subject areas
- Neuroscience(all)
Cite this
The translation of translational control by FMRP : Therapeutic targets for FXS. / Darnell, Jennifer C.; Klann, Eric.
In: Nature Neuroscience, Vol. 16, No. 11, 11.2013, p. 1530-1536.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - The translation of translational control by FMRP
T2 - Therapeutic targets for FXS
AU - Darnell, Jennifer C.
AU - Klann, Eric
PY - 2013/11
Y1 - 2013/11
N2 - De novo protein synthesis is necessary for long-lasting modifications in synaptic strength and dendritic spine dynamics that underlie cognition. Fragile X syndrome (FXS), characterized by intellectual disability and autistic behaviors, holds promise for revealing the molecular basis for these long-term changes in neuronal function. Loss of function of the fragile X mental retardation protein (FMRP) results in defects in synaptic plasticity and cognition in many models of the disease. FMRP is a polyribosome-associated RNA-binding protein that regulates the synthesis of a set of plasticity-reated proteins by stalling ribosomal translocation on target mRNAs. The recent identification of mRNA targets of FMRP and its upstream regulators, and the use of small molecules to stall ribosomes in the absence of FMRP, have the potential to be translated into new therapeutic avenues for the treatment of FXS.
AB - De novo protein synthesis is necessary for long-lasting modifications in synaptic strength and dendritic spine dynamics that underlie cognition. Fragile X syndrome (FXS), characterized by intellectual disability and autistic behaviors, holds promise for revealing the molecular basis for these long-term changes in neuronal function. Loss of function of the fragile X mental retardation protein (FMRP) results in defects in synaptic plasticity and cognition in many models of the disease. FMRP is a polyribosome-associated RNA-binding protein that regulates the synthesis of a set of plasticity-reated proteins by stalling ribosomal translocation on target mRNAs. The recent identification of mRNA targets of FMRP and its upstream regulators, and the use of small molecules to stall ribosomes in the absence of FMRP, have the potential to be translated into new therapeutic avenues for the treatment of FXS.
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UR - http://www.scopus.com/inward/citedby.url?scp=84886953546&partnerID=8YFLogxK
U2 - 10.1038/nn.3379
DO - 10.1038/nn.3379
M3 - Article
C2 - 23584741
AN - SCOPUS:84886953546
VL - 16
SP - 1530
EP - 1536
JO - Nature Neuroscience
JF - Nature Neuroscience
SN - 1097-6256
IS - 11
ER -