The DOI matches its Crossref record; 102 of the 102 references it cites were found in a registry or an index.
Reversal of Disease-Related Pathologies in the Fragile X Mouse Model by Selective Activation of GABA B Receptors with Arbaclofen
Open accessAbstract
Pharmacological activation of the GABA B receptor with arbaclofen in a mouse model of fragile X syndrome corrects neuronal defects associated with the disease.
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In the citing paper
“Activation of the GABA B receptor by arbaclofen decreased mRNA translation in the cortex of fragile X mice and corrected the increased spine density 78 .”
From Progress toward treatments for synaptic defects in autism (2013) · cites this paper as [78]
In the citing paper
“Administration of GABAB agonists, such as baclofen or arbaclofen, corrects exacerbated protein synthesis and multiple phenotypes in Fmr1-KO mice37–39.”
From Drug development for neurodevelopmental disorders: lessons learned from fragile X syndrome (2018) · cites this paper as [37]
In the citing paper
“treatment with mGluR antagonists (mavoglurant) and GABAB agonists (arbaclofen), as well as gene reinstatement in Fmr1-KO mice starting in young adult or adult mice, can fully correct most behavioural, physiological, biochemical and neuroanatomical alterations30,32,37.”
From Drug development for neurodevelopmental disorders: lessons learned from fragile X syndrome (2018) · cites this paper as [37]
In the citing paper
“No human data are available for SCA1 in the absence of a drug suitable for clinical trials30,32,37.”
From Drug development for neurodevelopmental disorders: lessons learned from fragile X syndrome (2018) · cites this paper as [37]
In the citing paper
“For many subtypes of ASD, there is a GABAB deficit and arbaclofen has rescued the behavioral deficits including social deficits in the mouse models of idiopathic ASD [127], deletion of 16p11.2 [128], and FXS [129].”
From An Update on Psychopharmacological Treatment of Autism Spectrum Disorder (2022) · cites this paper as [129]
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Citations
Cites
Malenka, R. C., & Bear, M. F. (2004) · Neuron · doi:10.1016/j.neuron.2004.09.012 · Cited by 3,968
Self-citationVerkerk, A. J. M. H., Pieretti, M., Sutcliffe, J. S., Fu, Y.-H., Kuhl, D. P. A., Pizzuti, A., Reiner, O., Richards, S., Victoria, M. F., Zhang, F., et al. (1991) · Cell · doi:10.1016/0092-8674(91)90397-h · Cited by 3,481
Self-citationFMRP Stalls Ribosomal Translocation on mRNAs Linked to Synaptic Function and Autism
Darnell, J. C., Van Driesche, S. J., Zhang, C., Hung, K. Y. S., Mele, A., Fraser, C. E., Stone, E. F., Chen, C., Fak, J. J., Chi, S. W., et al. (2011) · Cell · doi:10.1016/j.cell.2011.06.013 · Cited by 2,072
Kaech, S., & Banker, G. A. (2006) · Nature Protocols · doi:10.1038/nprot.2006.356 · Cited by 1,766
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
Self-citationAltered 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
Self-citationMorphometric study of human cerebral cortex development
Huttenlocher, P. R. (1990) · Neuropsychologia · doi:10.1016/0028-3932(90)90031-i · Cited by 1,239
Culturing hippocampal neurons.
Tanaka, H. (2002) · Folia Pharmacologica Japonica · doi:10.1254/fpj.119.163 · Cited by 1,141
Kanai, Y., Dohmae, N., & Hirokawa, N. (2004) · Neuron · doi:10.1016/j.neuron.2004.07.022 · Cited by 1,109
Fmr1 knockout mice: A model to study fragile X mental retardation
THEDUTCHBELGIANFRAGILEXCONSOR (1994) · Cell · doi:10.1016/0092-8674(94)90569-x · Cited by 1,108
Cited by
Sorted by citations
Hagerman, R. J., Berry-Kravis, E., Hazlett, H. C., Bailey, D. B., Moine, H., Kooy, R. F., Tassone, F., Gantois, I., Sonenberg, N., Mandel, J. L., et al. (2017) · Nature Reviews Disease Primers · doi:10.1038/nrdp.2017.65 · Cited by 838
Jiang, Y.-H., Yuen, R. K. C., Jin, X., Wang, M., Chen, N., Wu, X., Ju, J., Mei, J., Shi, Y., He, M., et al. (2013) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2013.06.012 · Cited by 474
The translation of translational control by FMRP: therapeutic targets for FXS
Darnell, J. C., & Klann, E. (2013) · Nature Neuroscience · doi:10.1038/nn.3379 · Cited by 491
mTOR complexes in neurodevelopmental and neuropsychiatric disorders
Costa-Mattioli, M., & Monteggia, L. M. (2013) · Nature Neuroscience · doi:10.1038/nn.3546 · Cited by 367
Drug development for neurodevelopmental disorders: lessons learned from fragile X syndrome
Berry-Kravis, E. M., Lindemann, L., Jønch, A. E., Apostol, G., Bear, M. F., Carpenter, R. L., Crawley, J. N., Curie, A., Des Portes, V., Hossain, F., et al. (2018) · Nature Reviews Drug Discovery · doi:10.1038/nrd.2017.221 · Cited by 336
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Metadata
- Authors
- Henderson, C., Wijetunge, L., Kinoshita, M. N., Shumway, M., Hammond, R. S., Postma, F. R., Brynczka, C., Rush, R., Thomas, A., Paylor, R., Warren, S. T., Vanderklish, P. W., Kind, P. C., Carpenter, R. L., Bear, M. F., & Healy, A. M.
- Affiliations
- Seaside Therapeutics (United States) (US)
- University of Edinburgh (GB)
- Emory University (US)
- Baylor College of Medicine (US)
- Scripps Research Institute (US)
- Massachusetts Institute of Technology (US)
- Corresponding author
- Healy, Aileen M.
- Venue
- Science Translational Medicine
- Published
- 2012-09-19
- Volume
- 4
- Issue
- 152
- Pages
- 152ra128-152ra128
- ISSN
- 1946-6234 (ISSN-L)
- 1946-6242
- PMC
- PMC8826584
- OpenAlex
- W2096270532
- Cited by
- 249
- References
- 102
- Citations by year
- 2026: 7 · 2025: 9 · 2024: 9 · 2023: 8 · 2022: 20 · 2021: 21 · 2020: 10 · 2019: 20 · 2018: 8 · 2017: 32 · 2016: 14 · 2015: 25 · 2014: 24 · 2013: 31 · 2012: 11
- FWCI
- 17.48
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Science Translational Medicine (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (HD 24064, HD020521, R01HD020521, P30HD024064, R37HD020521)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Neuroscience and Neuropharmacology Research
- Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Down syndrome and intellectual disability research
- Keywords
- fragile X syndrome, GABAB receptors, baclofen, Fmr1 knockout mice, FMRP, synaptic protein synthesis, dendritic spine morphology, AMPA receptor internalization, mRNA translation, synaptic dysfunction, intellectual disability, autism spectrum disorder, hippocampus
- MeSH terms
- Animals, Baclofen, Behavior, Animal, Disease Models, Animal, Fragile X Syndrome, Hippocampus, Male, Phenotype, Polyribosomes, Seizures, Synapses, Protein Biosynthesis, Receptors, GABA-B, Receptors, AMPA, Mice, Knockout, Protein Transport, Dendritic Spines, Mice, Fragile X Messenger Ribonucleoprotein 1, GABA-B Receptor Agonists, Drinking Water
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