The DOI matches its Crossref record; 69 of the 69 references it cites were found in a registry or an index.
Abnormal Striatal GABA Transmission in the Mouse Model for the Fragile X Syndrome
Open accessAbstract
Background Structural and functional neuroimaging studies suggest abnormal activity in the striatum of patients with the fragile X syndrome (FXS), the most common form of inherited mental retardation.Methods Neurophysiological and immunofluorescence experiments in striatal brain slices. We studied the synaptic transmission in a mouse model for FXS, as well as the subcellular localization of fragile X mental retardation protein (FMRP) and brain cytoplasmic (BC1) RNA in striatal axons.Results Our results show that absence of FMRP is associated with apparently normal striatal glutamate-mediated transmission, but abnormal gamma-aminobutyric acid (GABA) transmission. This effect is likely secondary to increased transmitter release from GABAergic nerve terminals. We detected the presence of FMRP in axons of striatal neurons and observed a selective increase in the frequency of spontaneous and miniature inhibitory postsynaptic currents (sIPSCs, mIPSCs) in fmr1-knockout mice. We also observed reduced paired-pulse ratio of evoked IPSCs, a finding that is consistent with the idea that transmitter release probability from striatal GABAergic nerve terminals is higher than normal in these mutants. Finally, we have identified the small noncoding BC1 RNA as a critical coplayer of FMRP in the regulation of striatal synaptic transmission.Conclusions Understanding the physiologic action of FMRP and the synaptic defects associated with GABA transmission might be useful to design appropriate pharmacologic interventions for FXS.
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In the citing paper
“The GABA receptor system has also been reported as downregulated in FXS, partially due to destabilization of the mRNAs encoding the GABA receptor subunits (76,80,113).”
From Fragile X syndrome: causes, diagnosis, mechanisms, and therapeutics (2012) · cites this paper as [76]
Experimental — accuracy not yet measuredIn the citing paper
“FMRP activity is regulated in response to metabotropic glutamate receptors (mGluRs) (78), 2-amino-3-(5-methyl-3-oxo-1,2-oxazol-4-yl) propanoic acid (AMPA) receptors (79), γ-aminobutyric acid (GABA) receptors (76,80,81), N-methyl-d-aspartate (NMDA) receptors (82)(83)(84), and the tyrosine kinase or BDNF/NT-3 growth factor (TrkB) receptors (85).”
From Fragile X syndrome: causes, diagnosis, mechanisms, and therapeutics (2012) · cites this paper as [76]
In the citing paper
“Furthermore, extensive electrophysiological studies in the Fmr1 KO mouse model indicated an excitation/inhibition (glutamate/GABA; see below) imbalance (75)(76)(77).”
From Fragile X syndrome: causes, diagnosis, mechanisms, and therapeutics (2012) · cites this paper as [76]
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Cites
Instability of a 550-Base Pair DNA Segment and Abnormal Methylation in Fragile X Syndrome
Oberlé, I., Rousseau, F., Heitz, D., Kretz, C., Devys, D., Hanauer, A., Boué, J., Bertheas, M. F., & Mandel, J. L. (1991) · Science · doi:10.1126/science.252.5009.1097 · Cited by 1,499
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
Brown, V., Jin, P., Ceman, S., Darnell, J. C., O'Donnell, W. T., Tenenbaum, S. A., Jin, X., Feng, Y., Wilkinson, K. D., Keene, J. D., et al. (2001) · Cell · doi:10.1016/s0092-8674(01)00568-2 · 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
Fragile X mental retardation protein targets G quartet mRNAs important for neuronal function
Darnell, J. C., Jensen, K. B., Jin, P., Brown, V., Warren, S. T., & Darnell, R. B. (2001) · Cell · doi:10.1016/s0092-8674(01)00566-9 · Cited by 952
Inhibitory control of neostriatal projection neurons by GABAergic interneurons
Koós, T. Z., & Tepper, J. M. (1999) · Nature Neuroscience · doi:10.1038/8138 · Cited by 863
Zalfa, F., Giorgi, M., Primerano, B., Moro, A., Di Penta, A., Reis, S., Oostra, B., & Bagni, C. (2003) · Cell · doi:10.1016/s0092-8674(03)00079-5 · Cited by 676
Self-citationNeuronal RNA Granules: Movers and Makers
Kiebler, M. A., & Bassell, G. J. (2006) · Neuron · doi:10.1016/j.neuron.2006.08.021 · Cited by 595
Krichevsky, A. M., & Kosik, K. S. (2001) · Neuron · doi:10.1016/s0896-6273(01)00508-6 · Cited by 536
Wilson, C. J., & Groves, P. M. (1980) · The Journal of Comparative Neurology · doi:10.1002/cne.901940308 · Cited by 527
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Interneuron dysfunction in psychiatric disorders
Marín, O. (2012) · Nature Reviews Neuroscience · doi:10.1038/nrn3155 · Cited by 1,190
Fragile X Syndrome: Loss of Local mRNA Regulation Alters Synaptic Development and Function
Bassell, G. J., & Warren, S. T. (2008) · Neuron · doi:10.1016/j.neuron.2008.10.004 · Cited by 1,051
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
The Fragile X Syndrome Protein Represses Activity-Dependent Translation through CYFIP1, a New 4E-BP
Napoli, I., Mercaldo, V., Boyl, P. P., Eleuteri, B., Zalfa, F., De Rubeis, S., Di Marino, D., Mohr, E., Massimi, M., Falconi, M., et al. (2008) · Cell · doi:10.1016/j.cell.2008.07.031 · Cited by 609
Self-citationMolecular Mechanisms of Fragile X Syndrome: A Twenty-Year Perspective
Santoro, M. R., Bray, S. M., & Warren, S. T. (2012) · Annual Review of Pathology: Mechanisms of Disease · doi:10.1146/annurev-pathol-011811-132457 · Cited by 528
Metadata
- Authors
- Centonze, D., Rossi, S., Mercaldo, V., Napoli, I., Ciotti, M. T., De Chiara, V., Musella, A., Prosperetti, C., Calabresi, P., Bernardi, G., & Bagni, C.
- Affiliations
- University of Rome Tor Vergata (IT)
- University of Perugia (IT)
- ORCID
- Venue
- Biological Psychiatry
- Publisher
- Elsevier BV
- Published
- 2008-05
- Volume
- 63
- Issue
- 10
- Pages
- 963-973
- ISSN
- 0006-3223 (ISSN-L)
- OpenAlex
- W2012460847
- Cited by
- 171
- References
- 68
- Citations by year
- 2026: 4 · 2025: 2 · 2024: 4 · 2023: 10 · 2022: 6 · 2021: 5 · 2020: 11 · 2019: 7 · 2018: 10 · 2017: 8 · 2016: 9 · 2015: 7 · 2014: 15 · 2013: 11 · 2012: 15
- FWCI
- 4.96
- Type
- journal article
- Language
- en
- Open access
- green
- lirias.kuleuven.be
- Licence
- Locations
- Biological Psychiatry (publishedVersion)
- PubMed (publishedVersion)
- Lirias (KU Leuven) (submittedVersion, other-oa)
- Cineca Institutional Research Information System (Tor Vergata University) (submittedVersion)
- Funders
- Ministero della Salute
- 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, BC1 RNA, striatum, GABAergic neurotransmission, inhibitory postsynaptic currents, miniature IPSCs, paired-pulse ratio, Fmr1 knockout mice, intellectual disability, synaptic plasticity
- MeSH terms
- Animals, Cerebral Cortex, Corpus Striatum, Disease Models, Animal, Electric Stimulation, Fragile X Syndrome, gamma-Aminobutyric Acid, Gene Expression Regulation, Mice, Inbred C57BL, Neural Inhibition, Synaptic Transmission, Phalloidine, Mice, Knockout, Patch-Clamp Techniques, Ribonucleoproteins, Small Cytoplasmic, RNA, Untranslated, Vesicular Inhibitory Amino Acid Transport Proteins, Mice, Fragile X Messenger Ribonucleoprotein 1, RNA, Long Noncoding, In Vitro Techniques
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