A novel function for fragile X mental retardation protein in translational activation
Open accessAbstract
Fragile X syndrome, the most frequent form of inherited mental retardation, is due to the absence of Fragile X Mental Retardation Protein (FMRP), an RNA-binding protein involved in several steps of RNA metabolism. To date, two RNA motifs have been found to mediate FMRP/RNA interaction, the G-quartet and the "kissing complex," which both induce translational repression in the presence of FMRP. We show here a new role for FMRP as a positive modulator of translation. FMRP specifically binds Superoxide Dismutase 1 (Sod1) mRNA with high affinity through a novel RNA motif, SoSLIP (Sod1 mRNA Stem Loops Interacting with FMRP), which is folded as three independent stem-loop structures. FMRP induces a structural modification of the SoSLIP motif upon its interaction with it. SoSLIP also behaves as a translational activator whose action is potentiated by the interaction with FMRP. The absence of FMRP results in decreased expression of Sod1. Because it has been observed that brain metabolism of FMR1 null mice is more sensitive to oxidative stress, we propose that the deregulation of Sod1 expression may be at the basis of several traits of the physiopathology of the Fragile X syndrome, such as anxiety, sleep troubles, and autism.
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FMRP is not only involved in translational repression, it is also a translational enhancer.
“Indeed, FMRP is not only involved in translational repression, it is also a translational enhancer (Bechara et al., 2009; Gross et al., 2011; Kwan et al., 2012; Tabet et al., 2016)”
From New insights into the regulatory function of CYFIP1 in the context of WAVE- and FMRP-containing complexes (2017) · cites this paper as Bechara et al., 2009
The absence of FMRP, as shown in FMR1-knockout mice, leads to increased oxidative stress (152-154).
“the absence of FMRP, as shown in FMR1-knockout mice, leads to increased oxidative stress (152–154).”
From Gene × Environment Interactions in Autism Spectrum Disorders: Role of Epigenetic Mechanisms (2014) · cites this paper as [152]
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Analysis of Relative Gene Expression Data Using Real-Time Quantitative PCR and the 2−ΔΔCT Method
Livak, K. J., & Schmittgen, T. D. (2001) · Methods · doi:10.1006/meth.2001.1262 · Cited by 180,860 · see citing works
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 · see citing works
AU-rich-element-mediated upregulation of translation by FXR1 and Argonaute 2
Vasudevan, S., & Steitz, J. A. (2007) · Cell · doi:10.1016/j.cell.2007.01.038 · Cited by 656 · see citing works
Evidence that fragile X mental retardation protein is a negative regulator of translation
Laggerbauer, B. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.4.329 · Cited by 571 · see citing works
Feng, Y., Absher, D., Eberhart, D. E., Brown, V., Malter, H. E., & Warren, S. T. (1997) · Molecular Cell · doi:10.1016/s1097-2765(00)80012-x · Cited by 511 · see citing works
RNA cargoes associating with FMRP reveal deficits in cellular functioning in Fmr1 null mice
Miyashiro, K. Y., Beckel-Mitchener, A., Purk, T. P., Becker, K. G., Barret, T., Liu, L., Carbonetto, S., Weiler, I. J., Greenough, W. T., & Eberwine, J. (2003) · Neuron · doi:10.1016/s0896-6273(03)00034-5 · Cited by 495 · see citing works
Fragile X Syndrome: Diagnosis, Treatment, And Research
Silverman, & Cronister, A. (1991) · Johns Hopkins University Press eBooks · Cited by 481 · see citing works
The fragile X mental retardation protein binds specifically to its mRNA via a purine quartet motif
Schaeffer, C., Bardoni, B., Mandel, J., Ehresmann, B., Ehresmann, C., & Moine, H. (2001) · The EMBO Journal · doi:10.1093/emboj/20.17.4803 · Cited by 471 · see citing works
Self-citationA new function for the fragile X mental retardation protein in regulation of PSD-95 mRNA stability
Zalfa, F., Eleuteri, B., Dickson, K. S., Mercaldo, V., Rubeis, S. D., di Penta, A., Tabolacci, E., Chiurazzi, P., Neri, G., Grant, S. G. N., et al. (2007) · Nature Neuroscience · doi:10.1038/nn1893 · Cited by 364 · see citing works
Lu, R., Wang, H., Liang, Z., Ku, L., O'Donnell, W. T., Li, W., Warren, S. T., & Feng, Y. (2004) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0404995101 · Cited by 322 · see citing works
Darnell, J. C., Fraser, C. E., Mostovetsky, O., Stefani, G., Jones, T. A., Eddy, S. R., & Darnell, R. B. (2005) · Genes & Development · doi:10.1101/gad.1276805 · Cited by 287 · see citing works
Todd, P. K., Mack, K. J., & Malter, J. S. (2003) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.2336265100 · Cited by 280 · see citing works
Fragile X mental retardation protein is associated with translating polyribosomes in neuronal cells
Stefani, G., Fraser, C. E., Darnell, J. C., & Darnell, R. B. (2004) · The Journal of Neuroscience · doi:10.1523/jneurosci.2306-04.2004 · Cited by 274 · see citing works
Juarez, J. C., Manuia, M., Burnett, M. E., Betancourt, O., Boivin, B., Shaw, D. E., Tonks, N. K., Mazar, A. P., & Doñate, F. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0709451105 · Cited by 255 · see citing works
Binder, M. D., Hirokawa, N., & Windhorst, U. (2008) · Springer eBooks · doi:10.1007/978-3-540-29678-2_3942 · Cited by 245 · see citing works
Increased excretion of a lipid peroxidation biomarker in autism
Ming, X., Stein, T. P., Brimacombe, M., Johnson, W. G., Lambert, G. H., & Wagner, G. C. (2005) · Prostaglandins, Leukotrienes and Essential Fatty Acids · doi:10.1016/j.plefa.2005.06.002 · Cited by 236 · see citing works
Corbin, F. (1997) · Human Molecular Genetics · doi:10.1093/hmg/6.9.1465 · Cited by 235 · 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
Khandjian, E. W., Huot, M.-E., Tremblay, S., Davidovic, L., Mazroui, R., & Bardoni, B. (2004) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0405398101 · Cited by 178 · see citing works
Self-citationOxidative stress is the new stress
Gingrich, J. A. (2005) · Nature Medicine · doi:10.1038/nm1205-1281 · Cited by 163 · see citing works
FMRP interferes with the Rac1 pathway and controls actin cytoskeleton dynamics in murine fibroblasts
Castets, M., Schaeffer, C., Bechara, E., Schenck, A., Khandjian, E. W., Luche, S., Moine, H., Rabilloud, T., Mandel, J.-L., & Bardoni, B. (2005) · Human Molecular Genetics · doi:10.1093/hmg/ddi077 · Cited by 162 · see citing works
Self-citationTransport of Fragile X Mental Retardation Protein via Granules in Neurites of PC12 Cells
de Diego‐Otero, Y., Severijnen, L.-A. W. F. M., van Cappellen, G. W. A., Schrier, M., Oostra, B. A., & Willemsen, R. (2002) · Molecular and Cellular Biology · doi:10.1128/mcb.22.23.8332-8341.2002 · Cited by 149 · 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
[1] Probing RNA structure and RNA-ligand complexes with chemical probes
Brunel, C., & Romby, P. (2000) · Methods in enzymology on CD-ROM/Methods in enzymology · doi:10.1016/s0076-6879(00)18040-1 · Cited by 135 · see citing works
Davidovic, L., Jaglin, X. H., Lepagnol-Bestel, A.-M., Tremblay, S., Simonneau, M., Bardoni, B., & Khandjian, E. W. (2007) · Human Molecular Genetics · doi:10.1093/hmg/ddm263 · Cited by 134 · see citing works
Self-citationde Diego-Otero, Y., Romero-Zerbo, Y., Bekay, R. E., Decara, J., Sanchez, L., Fonseca, F. R.-D., & Arco-Herrera, I. D. (2009) · Neuropsychopharmacology · doi:10.1038/npp.2008.152 · Cited by 133 · see citing works
FMRP RNA targets: identification and validation
Darnell, J. C., Mostovetsky, O., & Darnell, R. B. (2005) · Genes Brain & Behavior · doi:10.1111/j.1601-183x.2005.00144.x · Cited by 132 · see citing works
The fragile X syndrome: exploring its molecular basis and seeking a treatment
Bardoni, B., Davidovic, L., Bensaid, M., & Khandjian, É. W. (2006) · Expert Reviews in Molecular Medicine · doi:10.1017/s1462399406010751 · Cited by 100 · see citing works
Self-citationAberrant early-phase ERK inactivation impedes neuronal function in fragile X syndrome
Kim, S. H., Markham, J. A., Weiler, I. J., & Greenough, W. T. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0800257105 · Cited by 97 · see citing works
Adinolfi, S., Bagni, C., Musco, G., Gibson, T. J., Mazzarella, L., & Pastore, A. (1999) · RNA · doi:10.1017/s1355838299990647 · Cited by 96 · see citing works
Fragile X Syndrome: Diagnosis, Treatment, and Research
Schmidt, M. A. (1997) · JAMA · doi:10.1001/jama.1997.03540380083039 · Cited by 95 · see citing works
El Bekay, R., Romero‐Zerbo, Y., Decara, J., Sanchez‐Salido, L., Del Arco‐Herrera, I., Rodríguez‐de Fonseca, F., & De Diego‐Otero, Y. (2007) · European Journal of Neuroscience · doi:10.1111/j.1460-9568.2007.05939.x · Cited by 85 · see citing works
On BC1 RNA and the fragile X mental retardation protein
Iacoangeli, A., Rozhdestvensky, T. S., Dolzhanskaya, N., Tournier, B., Schütt, J., Brosius, J., Denman, R. B., Khandjian, E. W., Kindler, S., & Tiedge, H. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0710991105 · Cited by 83 · see citing works
The Drosophila fragile X-related gene regulates axoneme differentiation during spermatogenesis
Zhang, Y., Matthies, H. J. G., Mancuso, J. J., Andrews, H. K., Woodruff, E. A., Friedman, D., & Broadie, K. S. (2004) · Developmental Biology · doi:10.1016/j.ydbio.2004.02.010 · Cited by 76 · see citing works
Zhang, Y., Friedman, D. B., Wang, Z., Woodruff, E. A., Pan, L., O’Donnell, J. M., & Broadie, K. S. (2005) · Molecular & Cellular Proteomics · doi:10.1074/mcp.m400174-mcp200 · Cited by 65 · see citing works
Bechara, E., Davidovic, L., Melko, M., Bensaid, M., Tremblay, S., Grosgeorge, J., Khandjian, E. W., Lalli, E., & Bardoni, B. (2006) · Nucleic Acids Research · doi:10.1093/nar/gkl1021 · Cited by 59 · see citing works
Self-citationDevelopment of a new bicistronic retroviral vector with strong IRES activity
Martin, P., Albagli, O., Poggi, M. C., Boulukos, K. E., & Pognonec, P. (2006) · BMC Biotechnology · doi:10.1186/1472-6750-6-4 · Cited by 55 · see citing works
Self-citationSleep disruption, oxidative stress, and aging: New insights from fruit flies
Cirelli, C. (2006) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0606652103 · Cited by 32 · see citing works
Fragile X Mental Retardation Protein: Many Partners and Multiple Targets for a Promiscuous Function
Khandjian, E. W., Bechara, E., Davidovic, L., & Bardoni, B. (2005) · Current Genomics · doi:10.2174/138920205775067701 · Cited by 10 · see citing works
Self-citation
Flagged first, then by citations
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Sorted by citations
FMRP 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 · see citing works
Global signatures of protein and mRNA expression levels
de Sousa Abreu, R., Penalva, L. O., Marcotte, E. M., & Vogel, C. (2009) · Molecular BioSystems · doi:10.1039/b908315d · Cited by 1,248 · see citing works
RNA-binding proteins in human genetic disease
Gebauer, F., Schwarzl, T., Valcárcel, J., & Hentze, M. W. (2020) · Nature Reviews Genetics · doi:10.1038/s41576-020-00302-y · Cited by 1,174 · see citing works
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 · see citing works
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 · see citing works
Energetics, epigenetics, mitochondrial genetics
Wallace, D. C., & Fan, W. (2009) · Mitochondrion · doi:10.1016/j.mito.2009.09.006 · Cited by 507 · see citing works
The Pathophysiology of Fragile X (and What It Teaches Us about Synapses)
Bhakar, A. L., Dölen, G., & Bear, M. F. (2012) · Annual Review of Neuroscience · doi:10.1146/annurev-neuro-060909-153138 · Cited by 370 · see citing works
Gene × Environment Interactions in Autism Spectrum Disorders: Role of Epigenetic Mechanisms
Tordjman, S., Somogyi, E., Coulon, N., Kermarrec, S., Cohen, D., Bronsard, G., Bonnot, O., Weismann-Arcache, C., Botbol, M., Lauth, B., et al. (2014) · Frontiers in Psychiatry · doi:10.3389/fpsyt.2014.00053 · Cited by 283 · see citing works
Millevoi, S., Moine, H., & Vagner, S. (2012) · Wiley Interdisciplinary Reviews - RNA · doi:10.1002/wrna.1113 · Cited by 277 · see citing works
Self-citationDisruption of RNA Metabolism in Neurological Diseases and Emerging Therapeutic Interventions
Nussbacher, J. K., Tabet, R., Yeo, G. W.-M., & Lagier‐Tourenne, C. (2019) · Neuron · doi:10.1016/j.neuron.2019.03.014 · Cited by 264 · see citing works
Smaga, I., Niedzielska, E., Gawlik, M., Moniczewski, A., Krzek, J., Przegaliński, E., Pera, J., & Filip, M. (2015) · Pharmacological Reports · doi:10.1016/j.pharep.2014.12.015 · Cited by 252 · see citing works
An epigenetic framework for neurodevelopmental disorders: From pathogenesis to potential therapy
Millan, M. J. (2013) · Neuropharmacology · doi:10.1016/j.neuropharm.2012.11.015 · Cited by 229 · see citing works
G‐quadruplexes: Emerging roles in neurodegenerative diseases and the non‐coding transcriptome
Simone, R., Fratta, P., Neidle, S., Parkinson, G. N., & Isaacs, A. M. (2015) · FEBS Letters · doi:10.1016/j.febslet.2015.05.003 · Cited by 216 · see citing works
The Unstable Repeats—Three Evolving Faces of Neurological Disease
Nelson, D. L., Orr, H. T., & Warren, S. T. (2013) · Neuron · doi:10.1016/j.neuron.2013.02.022 · Cited by 200 · see citing works
Valenti, D., Lidia de, B. A. R. I., Filippis, B. D., Henrion‐Caude, A., & Vacca, R. A. (2014) · Neuroscience & Biobehavioral Reviews · doi:10.1016/j.neubiorev.2014.01.012 · Cited by 189 · see citing works
Lozano, R., Rosero, C. A., & Hagerman, R. J. (2014) · Intractable & Rare Diseases Research · doi:10.5582/irdr.2014.01022 · Cited by 186 · see citing works
The neural correlates of inner speech defined by voxel-based lesion-symptom mapping
Geva, S., Jones, P. S., Crinion, J. T., Price, C. J., Baron, J.-C., & Warburton, E. A. (2011) · Brain · doi:10.1093/brain/awr232 · Cited by 173 · see citing works
Gross, C., Yao, X., Pong, D. L., Jeromin, A., & Bassell, G. J. (2011) · The Journal of Neuroscience · doi:10.1523/jneurosci.6661-10.2011 · Cited by 161 · see citing works
Schütt, J., Falley, K., Richter, D., Kreienkamp, H., & Kindler, S. (2009) · Journal of Biological Chemistry · doi:10.1074/jbc.m109.042663 · Cited by 155 · see citing works
RNA G-quadruplexes and their potential regulatory roles in translation
Song, J., Perreault, J., Topisirović, I., & Richard, S. (2016) · Translation · doi:10.1080/21690731.2016.1244031 · Cited by 153 · see citing works
Maurin, T., Lebrigand, K., Castagnola, S., Paquet, A. C., Jarjat, M., Popa, A., Grossi, M., Rage, F., & Bardoni, B. (2018) · Nucleic Acids Research · doi:10.1093/nar/gky267 · Cited by 152 · see citing works
Self-citationFragile X mental retardation 1 gene enhances the translation of large autism-related proteins
Greenblatt, E. J., & Spradling, A. C. (2018) · Science · doi:10.1126/science.aas9963 · Cited by 140 · see citing works
Species-dependent posttranscriptional regulation of NOS1 by FMRP in the developing cerebral cortex
Kwan, K. Y., Lam, M. M. S., Johnson, M. B., Dube, U., Shim, S., Rašin, M.-R., Sousa, A. M. M., Fertuzinhos, S., Chen, J.-G., Arellano, J. I., et al. (2012) · Cell · doi:10.1016/j.cell.2012.02.060 · Cited by 137 · see citing works
Kronk, R. A., Bishop, E. E., Raspa, M., Bickel, J., Mandel, D. A., & Bailey, D. B. (2010) · SLEEP · doi:10.1093/sleep/33.5.679 · Cited by 129 · see citing works
Zhang, Y., Brown, M. R., Hyland, C., Chen, Y., Kronengold, J., Fleming, M. R., Kohn, A. B., Moroz, L. L., & Kaczmarek, L. K. (2012) · Journal of Neuroscience · doi:10.1523/jneurosci.2162-12.2012 · Cited by 125 · see citing works
Mechanism-based approaches to treating fragile X
Dölen, G., Carpenter, R. L., Ocain, T. D., & Bear, M. F. (2010) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2010.02.008 · Cited by 124 · see citing works
Of Men and Mice: Modeling the Fragile X Syndrome
Dahlhaus, R. (2018) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2018.00041 · Cited by 118 · see citing works
New perspectives on the biology of fragile X syndrome
Wang, T., Bray, S. M., & Warren, S. T. (2012) · Current Opinion in Genetics & Development · doi:10.1016/j.gde.2012.02.002 · Cited by 116 · see citing works
A metabolomic and systems biology perspective on the brain of the fragile X syndrome mouse model
Davidovic, L., Navratil, V., Bonaccorso, C. M., Catania, M. V., Bardoni, B., & Dumas, M.-E. (2011) · Genome Research · doi:10.1101/gr.116764.110 · Cited by 118 · see citing works
Self-citationInduced pluripotent stem cells: a new revolution for clinical neurology?
Mattis, V. B., & Svendsen, C. N. (2011) · The Lancet Neurology · doi:10.1016/s1474-4422(11)70022-9 · Cited by 112 · see citing works
Shen, M., Wang, F., Li, M., Sah, N., Stockton, M. E., Tidei, J. J., Gao, Y., Korabelnikov, T., Kannan, S., Vevea, J., et al. (2019) · Nature Neuroscience · doi:10.1038/s41593-019-0338-y · Cited by 112 · see citing works
Fragile X Syndrome: from molecular pathology to therapy
Maurin, T., Zongaro, S., & Bardoni, B. (2014) · Neuroscience & Biobehavioral Reviews · doi:10.1016/j.neubiorev.2014.01.006 · Cited by 113 · see citing works
Self-citationRNA-binding proteins balance brain function in health and disease
Schieweck, R., Ninkovic, J., & Kiebler, M. A. (2020) · Physiological Reviews · doi:10.1152/physrev.00047.2019 · Cited by 104 · see citing works
The Emerging Field of Epitranscriptomics in Neurodevelopmental and Neuronal Disorders
Angelova, M. T., Dimitrova, D. G., Dinges, N., Lenče, T., Worpenberg, L., Carré, C., & Roignant, J. (2018) · Frontiers in Bioengineering and Biotechnology · doi:10.3389/fbioe.2018.00046 · Cited by 106 · see citing works
Therapeutic Strategies in Fragile X Syndrome: From Bench to Bedside and Back
Gross, C., Hoffmann, A., Bassell, G. J., & Berry-Kravis, E. M. (2015) · Neurotherapeutics · doi:10.1007/s13311-015-0355-9 · Cited by 105 · see citing works
Dahlhaus, R., & El-Husseini, A. (2010) · Behavioural Brain Research · doi:10.1016/j.bbr.2009.11.019 · Cited by 102 · see citing works
Post-transcriptional Stimulation of Gene Expression by MicroRNAs
Lee, S., & Vasudevan, S. (2012) · Advances in experimental medicine and biology · doi:10.1007/978-1-4614-5107-5_7 · Cited by 104 · see citing works
Therapeutic Strategies in Fragile X Syndrome: Dysregulated mGluR Signaling and Beyond
Groß, C., Berry‐Kravis, E., & Bassell, G. J. (2011) · Neuropsychopharmacology · doi:10.1038/npp.2011.137 · Cited by 103 · see citing works
ERK/MAPK signaling and autism spectrum disorders
Vithayathil, J., Pucilowska, J., & Landreth, G. E. (2018) · Progress in brain research · doi:10.1016/bs.pbr.2018.09.008 · Cited by 101 · see citing works
Deregulated mTOR-mediated translation in intellectual disability.
Troca-Marín, J. A., Alves-Sampaio, A., & Montesinos, M. (2012) · Progress in neurobiology · doi:10.1016/j.pneurobio.2012.01.005 · Cited by 97 · see citing works
Co-regulation of mRNA translation by TDP-43 and Fragile X Syndrome protein FMRP
Majumder, P., Chu, J.-F., Chatterjee, B., Swamy, K. B. S., & Shen, C.-K. J. (2016) · Acta Neuropathologica · doi:10.1007/s00401-016-1603-8 · Cited by 100 · see citing works
Gholizadeh, S., Halder, S. K., & Hampson, D. R. (2015) · Brain Research · doi:10.1016/j.brainres.2014.11.023 · Cited by 92 · see citing works
Dysregulation of protein synthesis and disease
Quesne, J. P. C. L., Spriggs, K. A., Bushell, M., & Willis, A. E. (2009) · The Journal of Pathology · doi:10.1002/path.2627 · Cited by 97 · see citing works
Lyon, E., Laver, T. A., Yu, P., Jama, M., Young, K., Zoccoli, M. A., & Marlowe, N. (2010) · Journal of Molecular Diagnostics · doi:10.2353/jmoldx.2010.090229 · Cited by 95 · see citing works
The GABAAreceptor is an FMRP target with therapeutic potential in fragile X syndrome
Braat, S., D’Hulst, C., Heulens, I., Rubeis, S. D., Mientjes, E., Nelson, D. L., Willemsen, R., Bagni, C., Dam, D. V., Deyn, P. P. D., et al. (2015) · Cell Cycle · doi:10.4161/15384101.2014.989114 · Cited by 92 · see citing works
Update on the implication of potassium channels in autism: K+ channelautism spectrum disorder
Guglielmi, L. (2015) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2015.00034 · Cited by 89 · see citing works
Melko, M., Douguet, D., Bensaid, M., Zongaro, S., Verheggen, C., Gécz, J., & Bardoni, B. (2011) · Human Molecular Genetics · doi:10.1093/hmg/ddr069 · Cited by 89 · see citing works
Self-citationModulation of dADAR-dependent RNA editing by the Drosophila fragile X mental retardation protein
Bhogal, B., Jepson, J. E. C., Savva, Y. A., Pepper, A. S.-R., Reenan, R. A., & Jongens, T. A. (2011) · Nature Neuroscience · doi:10.1038/nn.2950 · Cited by 87 · see citing works
Kalmbach, B. E., Johnston, D., & Brager, D. H. (2015) · eNeuro · doi:10.1523/eneuro.0114-15.2015 · Cited by 82 · see citing works
Hippocampal deficits in neurodevelopmental disorders
Li, Y., Shen, M., Stockton, M. E., & Zhao, X. (2018) · Neurobiology of Learning and Memory · doi:10.1016/j.nlm.2018.10.001 · Cited by 84 · see citing works
Zongaro, S., Hukema, R. K., D’Antoni, S., Davidovic, L., Barbry, P., Catania, M. V., Willemsen, R., Mari, B. P., & Bardoni, B. (2013) · Human Molecular Genetics · doi:10.1093/hmg/ddt044 · Cited by 77 · see citing works
Self-citationUversky, V. N. (2017) · Autophagy · doi:10.1080/15548627.2017.1384889 · Cited by 76 · see citing works
The RGG motif proteins: Interactions, functions, and regulations
Chowdhury, M. N., & Jin, H. (2022) · Wiley Interdisciplinary Reviews - RNA · doi:10.1002/wrna.1748 · Cited by 68 · see citing works
Neurodegenerative diseases: Quantitative predictions of protein–RNA interactions
Cirillo, D., Agostini, F., Klus, P., Marchese, D., Rodriguez-Mulero, S., Bolognesi, B., & Tartaglia, G. G. (2012) · RNA · doi:10.1261/rna.034777.112 · Cited by 74 · see citing works
The role of G-quadruplex in RNA metabolism: involvement of FMRP and FMR2P
Melko, M., & Bardoni, B. (2010) · Biochimie · doi:10.1016/j.biochi.2010.05.018 · Cited by 74 · see citing works
Self-citationFatimy, R. E., Tremblay, S., Dury, A. Y., Solomon, S., Koninck, P. D., Schrader, J. W., & Khandjian, É. W. (2012) · PLoS ONE · doi:10.1371/journal.pone.0039338 · Cited by 73 · see citing works
Intrinsic Disorder in Proteins with Pathogenic Repeat Expansions
Darling, A. L., & Uversky, V. N. (2017) · Molecules · doi:10.3390/molecules22122027 · Cited by 72 · see citing works
RNA-Binding Proteins Impacting on Internal Initiation of Translation
Martı́nez-Salas, E., Lozano, G., Fernández-Chamorro, J., Francisco‐Velilla, R., Galan, A., & Diaz, R. L. (2013) · International Journal of Molecular Sciences · doi:10.3390/ijms141121705 · Cited by 68 · see citing works
Channelopathies and dendritic dysfunction in fragile X syndrome
Brager, D. H., & Johnston, D. (2014) · Brain Research Bulletin · doi:10.1016/j.brainresbull.2014.01.002 · Cited by 64 · see citing works
Abekhoukh, S., Sahin, H. B., Grossi, M., Zongaro, S., Maurin, T., Madrigal, I., Kazue-Sugioka, D., Raas-Rothschild, A., Doulazmi, M., Carrera, P., et al. (2017) · Disease Models & Mechanisms · doi:10.1242/dmm.025809 · Cited by 59 · see citing works
“STEK cells and 14 DIV cultured cortical neurons, expressing or not FMRP (Bechara et al., 2009; Maurin et al., 2015) and expressing ShScr or Sh89, were seeded in 96-well plates (20,000 cells/well) and transfected with the pRLTK constructs (with or without a G-quadruplex RNA-forming structure on the…”
InfluentialSelf-citationIdentification of consensus binding sites clarifies FMRP binding determinants
Anderson, B. R., Chopra, P., Suhl, J. A., Warren, S. T., & Bassell, G. J. (2016) · Nucleic Acids Research · doi:10.1093/nar/gkw593 · Cited by 59 · see citing works
Costa, L., Sardone, L. M., Lacivita, E., Leopoldo, M., & Ciranna, L. (2015) · Frontiers in Behavioral Neuroscience · doi:10.3389/fnbeh.2015.00065 · Cited by 57 · see citing works
The Neurobiology of X-Linked Intellectual Disability
Bassani, S., Zapata, J., Gerosa, L., Moretto, E., Murru, L., & Passafaro, M. (2013) · The Neuroscientist · doi:10.1177/1073858413493972 · Cited by 54 · see citing works
Hyperexcitability and homeostasis in fragile X Syndrome.
Liu, X., Kumar, V., Tsai, N.-P., & Auerbach, B. D. (2022) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2021.805929 · Cited by 53 · see citing works
Gatto, C. L. (2009) · Frontiers in Neural Circuits · doi:10.3389/neuro.04.008.2009 · Cited by 50 · see citing works
Bonaccorso, C. M., Spatuzza, M., Di Marco, B., Gloria, A., Barrancotto, G., Cupo, A., Musumeci, S. A., D'Antoni, S., Bardoni, B., & Catania, M. V. (2015) · International Journal of Developmental Neuroscience · doi:10.1016/j.ijdevneu.2015.02.004 · Cited by 52 · see citing works
Self-citationRNA‐binding proteins involved in RNA localization and their implications in neuronal diseases
Tolino, M., Köhrmann, M., & Kiebler, M. (2012) · European Journal of Neuroscience · doi:10.1111/j.1460-9568.2012.08160.x · Cited by 42 · see citing works
Translation regulation of mRNAs by the fragile X family of proteins through the microRNA pathway
Cheever, A., & Ceman, S. (2009) · RNA Biology · doi:10.4161/rna.6.2.8196 · Cited by 49 · see citing works
RNA-binding proteins as molecular links between cancer and neurodegeneration
Campos‐Melo, D., Droppelmann, C. A., Volkening, K., & Strong, M. J. (2014) · Biogerontology · doi:10.1007/s10522-014-9531-2 · Cited by 49 · see citing works
m6A methylation: Critical roles in aging and neurological diseases
Fan, Y., Lv, X., Chen, Z., Peng, Y., & Zhang, M. (2023) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2023.1102147 · Cited by 48 · see citing works
FMRP regulates MFF translation to locally direct mitochondrial fission in neurons
Fenton, A. R., Peng, R., Bond, C., Hugelier, S., Lakadamyali, M., Chang, Y., Holzbaur, E. L. F., & Jongens, T. A. (2024) · Nature Cell Biology · doi:10.1038/s41556-024-01544-2 · Cited by 47 · see citing works
From FMRP Function to Potential Therapies for Fragile X Syndrome
Sethna, F., Moon, C., & Wang, H. (2013) · Neurochemical Research · doi:10.1007/s11064-013-1229-3 · Cited by 47 · see citing works
Papoulas, O., Monzo, K. F., Cantin, G. T., Ruse, C. I., Yates, J. R., Ryu, Y. H., & Sisson, J. C. (2010) · Development · doi:10.1242/dev.055046 · Cited by 47 · see citing works
Discovering the 3′ UTR-mediated regulation of alpha-synuclein
Marchese, D., Botta‐Orfila, T., Cirillo, D., Rodríguez, J. A., Livi, C. M., Fernández‐Santiago, R., Ezquerra, M., Martı́, M. J., Bechara, E., Tartaglia, G. G., et al. (2017) · Nucleic Acids Research · doi:10.1093/nar/gkx1048 · Cited by 47 · see citing works
Self-citationFragile X-related protein family: a double-edged sword in neurodevelopmental disorders and cancer
Majumder, M., Johnson, R. H., & Palanisamy, V. (2020) · Critical Reviews in Biochemistry and Molecular Biology · doi:10.1080/10409238.2020.1810621 · Cited by 46 · see citing works
Kwon, K. J., Lee, E., Kim, M. K., Jeon, S., Choi, Y. Y., Shin, C. Y., & Han, S. (2015) · Neuroscience · doi:10.1016/j.neuroscience.2015.05.079 · Cited by 46 · see citing works
Dumas, M., & Davidovic, L. (2015) · Journal of Neuroimmune Pharmacology · doi:10.1007/s11481-014-9578-5 · Cited by 46 · see citing works
Self-citationDendritic protein synthesis in the normal and diseased brain
Swanger, S., & Bassell, G. (2012) · Neuroscience · doi:10.1016/j.neuroscience.2012.12.003 · Cited by 46 · see citing works
Serum levels of SOD and risk of autism spectrum disorder: A case‐control study
Wang, L., Jia, J., Zhang, J., & Li, K. (2016) · International Journal of Developmental Neuroscience · doi:10.1016/j.ijdevneu.2016.04.004 · Cited by 41 · see citing works
D’Antoni, S., Lidia de, B. A. R. I., Valenti, D., Borro, M., Bonaccorso, C. M., Simmaco, M., Vacca, R. A., & Catania, M. V. (2019) · Biological Chemistry · doi:10.1515/hsz-2019-0221 · Cited by 40 · see citing works
Prieto, M., Folci, A., & Martin, S. S. (2019) · Molecular Psychiatry · doi:10.1038/s41380-019-0629-4 · Cited by 41 · see citing works
Donnard, E. R., Shu, H., & Garber, M. G. (2022) · PLoS Genetics · doi:10.1371/journal.pgen.1010221 · Cited by 39 · see citing works
Won, J., Jin, Y., Choi, J., Park, S., Lee, T. Y., Lee, S., Chang, K., & Hong, Y. (2017) · International Journal of Molecular Sciences · doi:10.3390/ijms18061314 · Cited by 36 · see citing works
Dettori, L. G., Torrejon, D., Chakraborty, A., Dutta, A., Mohamed, M., Papp, C., Kuznetsov, V. A., Sung, P., Feng, W., & Bah, A. (2021) · Frontiers in Molecular Biosciences · doi:10.3389/fmolb.2021.691694 · Cited by 36 · see citing works
A Comparative Perspective on Wnt/β-Catenin Signalling in Cell Fate Determination
Garcin, C. L., & Habib, S. J. (2017) · Results and problems in cell differentiation · doi:10.1007/978-3-319-53150-2_15 · Cited by 36 · see citing works
Fragile X syndrome and model organisms: identifying potential routes of therapeutic intervention
Bhogal, B., & Jongens, T. A. (2010) · Disease Models & Mechanisms · doi:10.1242/dmm.002006 · Cited by 34 · see citing works
Activity dependent CAM cleavage and neurotransmission
Conant, K. E., Allen, M., & Seung Taik, L. I. M. (2015) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2015.00305 · Cited by 32 · see citing works
FMRP regulates actin filament organization via the armadillo protein p0071
Nolze, A., Schneider, J., Keil, R., Lederer, M., Hüttelmaier, S., Kessels, M. M., Qualmann, B., & Hatzfeld, M. (2013) · RNA · doi:10.1261/rna.037945.112 · Cited by 33 · see citing works
Impaired GABA neural circuits are critical for fragile X syndrome.
Gao, F., Qi, L., Yang, Z., Yang, T., Zhang, Y., Xu, H., & Zhao, H. (2018) · Neural Plasticity · doi:10.1155/2018/8423420 · Cited by 30 · see citing works
Matić, K., Eninger, T., Bardoni, B., Davidovic, L., & Maček, B. (2014) · Journal of Proteome Research · doi:10.1021/pr5006372 · Cited by 33 · see citing works
Self-citationRousseau, F., Labelle, Y., Bussières, J., & Lindsay, C. (2011) · Europe PMC (PubMed Central) · Cited by 30 · see citing works
Bilinovich, S. M., Uhl, K. L., Lewis, K., Soehnlen, X., Williams, M. R., Vogt, D. L., Prokop, J. W., & Campbell, D. B. (2020) · Developmental Neuroscience · doi:10.1159/000513536 · Cited by 31 · see citing works
Molecular and Genetic Analysis of the Drosophila Model of Fragile X Syndrome
Tessier, C. R., & Broadie, K. S. (2011) · Results and problems in cell differentiation · doi:10.1007/978-3-642-21649-7_7 · Cited by 31 · see citing works
Oxidative Stress in DNA Repeat Expansion Disorders: A Focus on NRF2 Signaling Involvement
Rosa, P. L., Petrillo, S., Bertini, E., & Piemonte, F. (2020) · Biomolecules · doi:10.3390/biom10050702 · Cited by 29 · see citing works
New Insights Into the Role of Cav2 Protein Family in Calcium Flux Deregulation in Fmr1-KO Neurons
Castagnola, S., Delhaye, S., Folci, A., Paquet, A. C., Brau, F., Duprat, F., Jarjat, M., Grossi, M., Béal, M., Martin, S., et al. (2018) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2018.00342 · Cited by 28 · see citing works
Self-citationOhashi, R., & Shiina, N. (2020) · Biomolecules · doi:10.3390/biom10020167 · Cited by 28 · see citing works
Ciranna, L., & Costa, L. (2019) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2019.00524 · Cited by 28 · see citing works
Extensive cross-regulation of post-transcriptional regulatory networks in DrosophilaPreprint
Stoiber, M. H., Olson, S., May, G. E., Duff, M. O., Manent, J., Obar, R. A., Guruharsha, K. G., Bickel, P. J., Artavanis‐Tsakonas, S., Brown, J. B., et al. (2015) · Genome Research · doi:10.1101/gr.182675.114 · Cited by 28 · see citing works
Shen, Z., Liu, B., Wu, B., Zhou, H., Wang, X., Cao, J., Jiang, M., Zhou, Y., Guo, F., Xue, C., et al. (2021) · Communications Biology · doi:10.1038/s42003-021-02071-8 · Cited by 27 · see citing works
FMRP-Driven Neuropathology in Autistic Spectrum Disorder and Alzheimer's disease: A Losing Game
Bleuzé, L., Triaca, V., & Borreca, A. (2021) · Frontiers in Molecular Biosciences · doi:10.3389/fmolb.2021.699613 · Cited by 27 · see citing works
- Authors
- Bechara, E. G., Didiot, M. C., Melko, M., Davidovic, L., Bensaid, M., Martin, P., Castets, M., Pognonec, P., Khandjian, E. W., Moine, H., & Bardoni, B.
- Affiliations
- Centre National de la Recherche Scientifique (FR)
- Corresponding author
- Bardoni, Barbara
- Venue
- PLoS Biology
- Publisher
- Public Library of Science (PLoS)
- Published
- 2009-01-20
- Volume
- 7
- Issue
- 1
- Pages
- e1000016
- ISSN
- 1544-9173 (ISSN-L)
- 1545-7885
- PMC
- PMC2628407
- OpenAlex
- W2077205550
- Cited by
- 206
- References
- 39
- Citations by year
- 2026: 2 · 2025: 5 · 2024: 7 · 2023: 7 · 2022: 15 · 2021: 16 · 2020: 13 · 2019: 9 · 2018: 15 · 2017: 13 · 2016: 10 · 2015: 19 · 2014: 11 · 2013: 10 · 2012: 19
- FWCI
- 10.53
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- PLoS Biology (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- HAL (submittedVersion, other-oa)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion)
- Figshare (submittedVersion, cc-by)
- Funders
- Agence Nationale de la Recherche
- Institut National de la Santé et de la Recherche Médicale
- Centre National de la Recherche Scientifique
- Centre Hospitalier Universitaire de Nice
- Canadian Institutes of Health Research
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA and protein synthesis mechanisms
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA Research and Splicing
- Keywords
- FMRP, fragile X syndrome, translational activation, superoxide dismutase 1, RNA-binding proteins, RNA folding, protein synthesis inhibition, oxidative stress, autism spectrum disorder, anxiety, sleep disorders
- MeSH terms
- Superoxide Dismutase-1, Animals, Binding Sites, Brain, Fragile X Syndrome, Gene Expression Regulation, Humans, Mice, Mutant Strains, Polyribosomes, RNA, Messenger, Superoxide Dismutase, Protein Biosynthesis, Mice, Fragile X Mental Retardation Protein, Fragile X Messenger Ribonucleoprotein 1
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