Rescue of behavioral phenotype and neuronal protrusion morphology in Fmr1 KO mice
Open accessAbstract
Lack of fragile X mental retardation protein (FMRP) causes Fragile X Syndrome, the most common form of inherited mental retardation. FMRP is an RNA-binding protein and is a component of messenger ribonucleoprotein complexes, associated with brain polyribosomes, including dendritic polysomes. FMRP is therefore thought to be involved in translational control of specific mRNAs at synaptic sites. In mice lacking FMRP, protein synthesis-dependent synaptic plasticity is altered and structural malformations of dendritic protrusions occur. One hypothesized cause of the disease mechanism is based on exaggerated group I mGluR receptor activation. In this study, we examined the effect of the mGluR5 antagonist MPEP on Fragile X related behavior in Fmr1 KO mice. Our results demonstrate a clear defect in prepulse inhibition of startle in Fmr1 KO mice, that could be rescued by MPEP. Moreover, we show for the first time a structural rescue of Fragile X related protrusion morphology with two independent mGluR5 antagonists.
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Preclinical studies have primarily focused on mGluR5 signaling as potential targets for treatment in adult mice.
“Preclinical studies have primarily focused on mGluR5 signaling as potential targets for treatment in adult mice 99,110-113 .”
From Developmental timing and critical windows for the treatment of psychiatric disorders (2016) · cites this paper as [110]
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“Therefore, FMRP is thought to be involved in the transport and/or the regulation of local mRNA translation at synaptic sites (Bagni and Greenough, 2005; Miyashiro et al., 2003; Weiler et al., 1997, 2004).”
BackgroundAnalysis of neocortex in three males with the Fragile X syndrome
Hinton, V. J., Brown, W. T., Wisniewski, K., & Rudelli, R. D. (1991) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320410306 · Cited by 527 · see citing works
Suppression of two major Fragile X Syndrome mouse model phenotypes by the mGluR5 antagonist MPEP
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Careaga, M., Noyon, T., Basuta, K., Van de Water, J., Tassone, F., Hagerman, R. J., & Ashwood, P. (2014) · Journal of Neuroinflammation · doi:10.1186/1742-2094-11-110 · Cited by 20 · see citing works
- Authors
- de Vrij, F. M. S., Levenga, J., van der Linde, H. C., Koekkoek, S. K., De Zeeuw, C. I., Nelson, D. L., Oostra, B. A., & Willemsen, R.
- Affiliations
- Erasmus MC (NL)
- Baylor College of Medicine (US)
- Corresponding author
- Willemsen, Rob
- Venue
- Neurobiology of Disease
- Publisher
- Elsevier BV
- Published
- 2008-07
- Volume
- 31
- Issue
- 1
- Pages
- 127-132
- ISSN
- 0969-9961 (ISSN-L)
- PMC
- PMC2481236
- OpenAlex
- W1969498835
- Cited by
- 313
- References
- 7
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 6 · 2023: 3 · 2022: 7 · 2021: 7 · 2020: 12 · 2019: 6 · 2018: 8 · 2017: 20 · 2016: 15 · 2015: 14 · 2014: 26 · 2013: 30 · 2012: 41
- FWCI
- 22.28
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Neurobiology of Disease (publishedVersion)
- PubMed (publishedVersion)
- EUR Research Repository (Erasmus University Rotterdam) (publishedVersion)
- PubMed Central (acceptedVersion)
- EUR Research Repository (Erasmus University Rotterdam) (submittedVersion)
- RePub (Erasmus University Rotterdam) (submittedVersion, other-oa)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- RePub (Erasmus University Rotterdam) (submittedVersion)
- Funders
- FRAXA Research Foundation
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (R01 HD38038, P30HD024064, R01HD038038)
- 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 › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Keywords
- fragile X syndrome, FMRP, Fmr1 knockout mice, mGluR5 antagonist, MPEP, synaptic plasticity, translational regulation
- MeSH terms
- Animals, Behavior, Animal, Cells, Cultured, Fragile X Syndrome, Hippocampus, Imidazoles, Neurons, Phenotype, Pyridines, Reflex, Startle, Receptors, Metabotropic Glutamate, Mice, Knockout, Microscopy, Confocal, Excitatory Amino Acid Antagonists, Mice, Fragile X Messenger Ribonucleoprotein 1, Fragile X Mental Retardation Protein, Receptor, Metabotropic Glutamate 5
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