Cited by 385Genetics and Neurodevelopmental DisordersNeuroscience and Neuropharmacology ResearchRNA and protein synthesis mechanisms

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Hypersensitivity to mGluR5 and ERK1/2 Leads to Excessive Protein Synthesis in the Hippocampus of a Mouse Model of Fragile X Syndrome

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Osterweil, Emily K., Krueger, Dilja D., Reinhold, Kimberly, Bear, Mark F.·2010·The Journal of Neuroscience

DOI 10.1523/jneurosci.3888-10.2010PMC PMC3400430

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Abstract

Fragile X syndrome (FXS) is caused by loss of the FMR1 gene product FMRP (fragile X mental retardation protein), a repressor of mRNA translation. According to the metabotropic glutamate receptor (mGluR) theory of FXS, excessive protein synthesis downstream of mGluR5 activation causes the synaptic pathophysiology that underlies multiple aspects of FXS. Here, we use an in vitro assay of protein synthesis in the hippocampus of male Fmr1 knock-out (KO) mice to explore the molecular mechanisms involved in this core biochemical phenotype under conditions where aberrant synaptic physiology has been observed. We find that elevated basal protein synthesis in Fmr1 KO mice is selectively reduced to wild-type levels by acute inhibition of mGluR5 or ERK1/2, but not by inhibition of mTOR (mammalian target of rapamycin). The mGluR5-ERK1/2 pathway is not constitutively overactive in the Fmr1 KO, however, suggesting that mRNA translation is hypersensitive to basal ERK1/2 activation in the absence of FMRP. We find that hypersensitivity to ERK1/2 pathway activation also contributes to audiogenic seizure susceptibility in the Fmr1 KO. These results suggest that the ERK1/2 pathway, and other neurotransmitter systems that stimulate protein synthesis via ERK1/2, represent additional therapeutic targets for FXS.

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Authors: Osterweil, E. K., Krueger, D. D., Reinhold, K., & Bear, M. F.
Affiliations: Howard Hughes Medical Institute (US)
Corresponding author: Bear, Mark F.
ORCID: Osterweil, Emily K.
Reinhold, Kimberly
Bear, Mark F.
Venue: The Journal of Neuroscience
Publisher: Society for Neuroscience
Published: 2010-11-17
Volume: 30
Issue: 46
Pages: 15616-15627
ISSN: 0270-6474 (ISSN-L)
1529-2401
DOI: 10.1523/jneurosci.3888-10.2010
PMC: PMC3400430
OpenAlex: W2092491372
Cited by: 384
References: 81
Citations by year: 2026: 11 · 2025: 20 · 2024: 18 · 2023: 15 · 2022: 17 · 2021: 24 · 2020: 27 · 2019: 23 · 2018: 22 · 2017: 34 · 2016: 33 · 2015: 29 · 2014: 30 · 2013: 31 · 2012: 24
FWCI: 14.40
Type: journal article
Language: en
Open access: hybrid
doi.org
Licence: cc-by-nc-sa
creativecommons.org/licenses/by-nc-sa/4.0/ (vor, 181 d)
Locations: Journal of Neuroscience (publishedVersion, cc-by-nc-sa)
Europe PMC (PubMed Central) (publishedVersion)
PubMed Central (publishedVersion)
PubMed (publishedVersion)
DSpace@MIT (Massachusetts Institute of Technology) (submittedVersion, cc-by-nc)
Edinburgh Research Explorer (University of Edinburgh) (submittedVersion, other-oa)
Funders: Howard Hughes Medical Institute
FRAXA Research Foundation
Simons Foundation
National Institute of Mental Health
National Institute of Child Health and Human Development
Eunice Kennedy Shriver National Institute of Child Health and Human Development (R01HD046943)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Neuroscience and Neuropharmacology Research
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA and protein synthesis mechanisms
Keywords: fragile X syndrome, FMRP, mGluR5, ERK1/2, protein synthesis, hippocampus, metabotropic glutamate receptors, mRNA translation, Fmr1 knockout mice, audiogenic seizures, mTOR, therapeutic target
MeSH terms: Animals, Disease Models, Animal, Fragile X Syndrome, Hippocampus, Isoenzymes, Male, Mice, Inbred C57BL, Pyridines, Protein Biosynthesis, Up-Regulation, Receptors, Metabotropic Glutamate, Mice, Knockout, Mitogen-Activated Protein Kinase 1, Mitogen-Activated Protein Kinase 3, Mice, Fragile X Messenger Ribonucleoprotein 1, Fragile X Mental Retardation Protein, Receptor, Metabotropic Glutamate 5

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Hypersensitivity to mGluR5 and ERK1/2 Leads to Excessive Protein Synthesis in the Hippocampus of a Mouse Model of Fragile X Syndrome

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