Fragile X: translation in action
Open accessAbstract
Fragile X is a synapsopathy--a disorder of synaptic function and plasticity. Recent studies using mouse models of the disease suggest that the critical defect is altered regulation of synaptic protein synthesis. Various strategies to restore balanced synaptic protein synthesis have been remarkably successful in correcting widely varied mutant phenotypes in mice. Insights gained by the study of synaptic plasticity in animal models of fragile X have suggested novel therapeutic approaches, not only for human fragile X but also for autism and mental retardation of unknown etiology.
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Research into the pathogenesis of ASD has largely focused on understanding unifying biochemical mechanisms based on the hypothesis that ASD is a 'synaptopathy'.
“For more than a decade, research into the pathogenesis of ASD has largely focused on understanding unifying biochemical mechanisms based on the hypothesis that ASD is a ‘synaptopathy’ (Bear et al., 2008; Dölen and Bear, 2009; Grabrucker et al., 2011).”
From Parallel social information processing circuits are differentially impacted in autism (2020) · cites this paper as Bear et al., 2008
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Cites
Synaptic Activity and the Construction of Cortical Circuits
Katz, L. C., & Shatz, C. J. (1996) · Science · doi:10.1126/science.274.5290.1133 · Cited by 3,048 · see citing works
The mGluR theory of fragile X mental retardation
Bear, M. F., Huber, K. M., & Warren, S. T. (2004) · Trends in Neurosciences · doi:10.1016/j.tins.2004.04.009 · Cited by 1,580 · see citing works
Self-citationSynaptic Assembly of the Brain in the Absence of Neurotransmitter Secretion
Verhage, M., Maia, A. S., Plomp, J. J., Brussaard, A. B., Heeroma, J. H., Vermeer, H., Toonen, R. F. G., Hammer, R. E., van den Berg, T. K., Berg, et al. (2000) · Science · doi:10.1126/science.287.5454.864 · Cited by 1,431 · see citing works
Altered synaptic plasticity in a mouse model of fragile X mental retardation
Huber, K. M., Gallagher, S. M., Warren, S. T., & Bear, M. F. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.122205699 · Cited by 1,349 · see citing works
Self-citationFmr1 knockout mice: A model to study fragile X mental retardation
THEDUTCHBELGIANFRAGILEXCONSOR (1994) · Cell · doi:10.1016/0092-8674(94)90569-x · Cited by 1,075 · see citing works
Role for rapid dendritic protein synthesis in hippocampal mGluR-dependent long-term depression.
Huber, K., Kayser, M., & Bear, M. (2000) · Science · doi:10.1126/science.288.5469.1254 · Cited by 1,015 · see citing works
Self-citationThe Autistic Neuron: Troubled Translation?
Kelleher, R. J., & Bear, M. F. (2008) · Cell · doi:10.1016/j.cell.2008.10.017 · Cited by 598 · see citing works
Self-citation(1994) · PubMed · Cited by 565 · see citing works
Suppression of two major Fragile X Syndrome mouse model phenotypes by the mGluR5 antagonist MPEP
Yan, Q. J., Rammal, M., Tranfaglia, M., & Bauchwitz, R. P. (2005) · Neuropharmacology · doi:10.1016/j.neuropharm.2005.06.004 · Cited by 521 · see citing works
McBride, S. M. J., Choi, C. H., Wang, Y., Liebelt, D. A., Braunstein, E. M., Ferreiro, D., Sehgal, A., Siwicki, K. K., Dockendorff, T. C., Nguyen, H. T., et al. (2005) · Neuron · doi:10.1016/j.neuron.2005.01.038 · Cited by 473 · see citing works
How Monocular Deprivation Shifts Ocular Dominance in Visual Cortex of Young Mice
Frenkel, M. Y., & Bear, M. F. (2004) · Neuron · doi:10.1016/j.neuron.2004.12.003 · Cited by 412 · see citing works
Self-citationQin, M., Kang, J., Burlin, T. V., Jiang, C., & Smith, C. B. (2005) · The Journal of Neuroscience · doi:10.1523/jneurosci.0093-05.2005 · Cited by 263 · see citing works
Bidirectional synaptic plasticity: from theory to reality
Bear, M. F. (2003) · Philosophical Transactions of the Royal Society of London. Series B: Biological Sciences · doi:10.1098/rstb.2002.1255 · Cited by 260 · see citing works
Self-citationRaymond, C. R., Thompson, V. L., Tate, W., & Abraham, W. (2000) · Journal of Neuroscience · doi:10.1523/jneurosci.20-03-00969.2000 · Cited by 246 · see citing works
A phenotypic and molecular characterization of the <i>fmr1‐tm1Cgr</i> Fragile X mouse
Yan, Q. J., Asafo‐Adjei, P. K., Arnold, H. M., Brown, R. E., & Bauchwitz, R. P. (2004) · Genes, Brain and Behavior · doi:10.1111/j.1601-183x.2004.00087.x · Cited by 213 · see citing works
Vanderklish, P. W., & Edelman, G. M. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.032681099 · Cited by 194 · see citing works
Chuang, S.-C., Zhao, W., Bauchwitz, R., Yan, Q., Bianchi, R., & Wong, R. K. S. (2005) · Journal of Neuroscience · doi:10.1523/jneurosci.1777-05.2005 · Cited by 190 · see citing works
Dendritic spine abnormalities in the occipital cortex of C57BL/6Fmr1 knockout mice
McKinney, B. C., Grossman, A. W., Elisseou, N. M., & Greenough, W. T. (2005) · American Journal of Medical Genetics Part B: Neuropsychiatric Genetics · doi:10.1002/ajmg.b.30183 · Cited by 184 · see citing works
FMR1 Knockout mice: A model to study fragile X mental retardation
Oostra, B. A., Bakker, C. E., & Reyniers, E. (1994) · The American Journal of Human Genetics · Cited by 181 · see citing works
Local Protein Synthesis and Spine Morphogenesis: Fragile X Syndrome and Beyond
Grossman, A. W., Aldridge, G. M., Weiler, I. J., & Greenough, W. T. (2006) · Journal of Neuroscience · doi:10.1523/jneurosci.1790-06.2006 · Cited by 178 · see citing works
Aschrafi, A., Cunningham, B. A., Edelman, G. M., & Vanderklish, P. W. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0409803102 · Cited by 148 · see citing works
A Morphological Correlate of Synaptic Scaling in Visual Cortex
Wallace, W., & Bear, M. F. (2004) · Journal of Neuroscience · doi:10.1523/jneurosci.1110-04.2004 · Cited by 139 · see citing works
Self-citationTherapeutic implications of the mGluR theory of fragile X mental retardation
Bear, M. F. (2005) · Genes, Brain and Behavior · doi:10.1111/j.1601-183x.2005.00135.x · Cited by 137 · see citing works
Self-citationRequirement of Protein Synthesis for Group I mGluR-Mediated Induction of Epileptiform Discharges
Merlin, L. R., Bergold, P. J., & Wong, R. K. S. (1998) · Journal of Neurophysiology · doi:10.1152/jn.1998.80.2.989 · Cited by 99 · see citing works
The Fragile X mental retardation protein
Bardoni, B., Schenck, A., & Mandel, J. (2001) · Brain Research Bulletin · doi:10.1016/s0361-9230(01)00647-5 · Cited by 91 · see citing works
Rapid Ocular Dominance Plasticity Requires Cortical but Not Geniculate Protein Synthesis
Taha, S. A., & Stryker, M. P. (2002) · Neuron · doi:10.1016/s0896-6273(02)00673-6 · Cited by 91 · see citing works
Hübener, M. (2003) · Current Opinion in Neurobiology · doi:10.1016/s0959-4388(03)00102-8 · Cited by 90 · see citing works
Zho, W.-M., You, J.-L., Huang, C., & Hsu, K. (2002) · Journal of Neuroscience · doi:10.1523/jneurosci.22-20-08838.2002 · Cited by 81 · see citing works
Differential translation and fragile X syndrome
Vanderklish, P. W., & Edelman, G. M. (2005) · Genes Brain & Behavior · doi:10.1111/j.1601-183x.2005.00134.x · Cited by 81 · see citing works
How do memories leave their mark?
Bear, M. F. (1997) · Nature · doi:10.1038/385481a0 · Cited by 23 · see citing works
Self-citationDölen, G. (2008) · Cited by 1 · see citing works
Self-citation
Flagged first, then by citations
Research works printed but not linked: 1
- Kelleher RJ, Bear MF (2007). The autistic neuron. Cell (in press).
Cited by
Sorted by citations
Correction of Fragile X Syndrome in Mice
Dölen, G., Osterweil, E., Rao, B. S. S., Smith, G. B., Auerbach, B. D., Chattarji, S., & Bear, M. F. (2007) · Neuron · doi:10.1016/j.neuron.2007.12.001 · Cited by 979 · see citing works
Self-citationReversal of learning deficits in a Tsc2+/− mouse model of tuberous sclerosis
Ehninger, D., Han, S., Shilyansky, C., Zhou, Y., Li, W., Kwiatkowski, D. J., Ramesh, V., & Silva, A. J. (2008) · Nature Medicine · doi:10.1038/nm1788 · Cited by 879 · see citing works
The Central Dogma Decentralized: New Perspectives on RNA Function and Local Translation in Neurons
Holt, C. E., & Schuman, E. M. (2013) · Neuron · doi:10.1016/j.neuron.2013.10.036 · Cited by 525 · see citing works
Silverman, J. L., Tolu, S. S., Barkan, C. L., & Crawley, J. N. (2010) · Neuropsychopharmacology · doi:10.1038/npp.2009.201 · Cited by 438 · see citing works
Genetic and Epigenetic Networks in Intellectual Disabilities
van Bokhoven, H. (2011) · Annual Review of Genetics · doi:10.1146/annurev-genet-110410-132512 · Cited by 382 · see citing works
Autism: A “Critical Period” Disorder?
LeBlanc, J. J., & Fagiolini, M. (2011) · Neural Plasticity · doi:10.1155/2011/921680 · Cited by 301 · see citing works
Gross, C., Nakamoto, M., Yao, X., Chan, C.-B., Yim, S. Y., Ye, K., Warren, S. T., & Bassell, G. J. (2010) · The Journal of Neuroscience · doi:10.1523/jneurosci.0402-10.2010 · Cited by 241 · see citing works
Reversing Neurodevelopmental Disorders in Adults
Ehninger, D., Li, W., Fox, K., Stryker, M. P., & Silva, A. J. (2008) · Neuron · doi:10.1016/j.neuron.2008.12.007 · Cited by 196 · see citing works
Genetics of intellectual disability
Ropers, H. H. (2008) · Current Opinion in Genetics & Development · doi:10.1016/j.gde.2008.07.008 · Cited by 199 · see citing works
Fragile X: Leading the Way for Targeted Treatments in Autism
Wang, L. W., Berry-Kravis, E., & Hagerman, R. J. (2010) · Neurotherapeutics · doi:10.1016/j.nurt.2010.05.005 · Cited by 176 · see citing works
The Genetics of Autism: Key Issues, Recent Findings, and Clinical Implications
El-Fishawy, P., & State, M. W. (2010) · Psychiatric Clinics of North America · doi:10.1016/j.psc.2009.12.002 · Cited by 166 · see citing works
Min, W. W., Yuskaitis, C. J., Yan, Q., Sikorski, C., Chen, S., Jope, R. S., & Bauchwitz, R. P. (2009) · Neuropharmacology · doi:10.1016/j.neuropharm.2008.09.017 · Cited by 146 · see citing works
Low sociability in BTBR T+tf/J mice is independent of partner strain
Yang, M., Abrams, D. N., Zhang, J. Y., Weber, M. D., Katz, A. M., Clarke, A. M., Silverman, J. L., & Crawley, J. N. (2012) · Physiology & Behavior · doi:10.1016/j.physbeh.2011.12.025 · Cited by 112 · see citing works
Utari, A., Chonchaiya, W., Rivera, S. M., Schneider, A., Hagerman, R. J., Faradz, S. M. H., Ethell, I. M., & Nguyen, D. V. (2010) · American Journal on Intellectual and Developmental Disabilities · doi:10.1352/1944-7558-115.5.433 · Cited by 105 · see citing works
Parallel social information processing circuits are differentially impacted in autism
Lewis, E. M., Stein-O’Brien, G. L., Patino, A. V., Nardou, R., Grossman, C. D., Brown, M., Bangamwabo, B., Ndiaye, N., Giovinazzo, D., Dardani, I., et al. (2020) · Neuron · doi:10.1016/j.neuron.2020.10.002 · Cited by 72 · see citing works
Self-citation
- Authors
- Bear, M. F., Dölen, G., Osterweil, E., & Nagarajan, N.
- Affiliations
- Howard Hughes Medical Institute (US)
- Corresponding author
- Bear, Mark F
- Venue
- Neuropsychopharmacology
- Published
- 2007-10-17
- Volume
- 33
- Issue
- 1
- Pages
- 84-87
- ISSN
- 0893-133X (ISSN-L)
- 1740-634X
- PMC
- PMC4327813
- OpenAlex
- W2130745215
- Cited by
- 94
- References
- 31
- Citations by year
- 2025: 2 · 2024: 1 · 2022: 4 · 2021: 6 · 2020: 3 · 2019: 3 · 2018: 2 · 2017: 2 · 2016: 3 · 2015: 1 · 2014: 3 · 2013: 11 · 2012: 12
- FWCI
- 5.71
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Neuropsychopharmacology (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- FRAXA Research Foundation
- Simons Foundation
- National Institute of Mental Health (F30MH067431)
- National Eye Institute
- 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, synaptic plasticity, synaptic protein synthesis, mouse model, treatment options, autism spectrum disorder, intellectual disability, translational research
- MeSH terms
- Animals, Disease Models, Animal, Fragile X Syndrome, Humans, Neuronal Plasticity, Synapses, Receptors, Metabotropic Glutamate
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