The Fragile X Syndrome Protein FMRP Associates with BC1 RNA and Regulates the Translation of Specific mRNAs at Synapses
Open accessAbstract
The Fragile X syndrome, which results from the absence of functional FMRP protein, is the most common heritable form of mental retardation. Here, we show that FMRP acts as a translational repressor of specific mRNAs at synapses. Interestingly, FMRP associates not only with these target mRNAs, but also with the dendritic, non-translatable RNA BC1. Blocking of BC1 inhibits the interaction of FMRP with its target mRNAs. Furthermore, BC1 binds directly to FMRP and can also associate, in the absence of any protein, with the mRNAs regulated by FMRP. This suggests a mechanism where BC1 could determine the specificity of FMRP function by linking the regulated mRNAs and FMRP. Thus, when FMRP is not present, loss of translational repression of specific mRNAs at synapses could result in synaptic dysfunction phenotype of Fragile X patients.
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FMRP negatively regulates translation of specific mRNAs at the synapse.
“This protein negatively regulates the translation of specific mRNAs at the synapse23,54.”
From Activity-dependent neuronal signalling and autism spectrum disorder (2013) · cites this paper as [54]
Absence of FMRP leads to increased local protein synthesis, initially shown for Arc, Map1B, and αCaMKII proteins.
“Absence of FMRP leads to an increase of local protein synthesis (Figures 2 and 3), initially shown for the activity-regulated cytoskeleton protein (Arc), microtubule-associated protein 1B (Map1B), and alpha Ca 2+ /calmodulin-dependent kinase II (αCaMKII) proteins (69).”
From Fragile X syndrome: causes, diagnosis, mechanisms, and therapeutics (2012) · cites this paper as [69]
Neurons in Fragile X ectopically overexpress Arc.
“As in oligomer-exposed neurons, neurons in Fragile X ectopically overexpress Arc (Zalfa et al., 2003).”
From The Aβ oligomer hypothesis for synapse failure and memory loss in Alzheimer’s disease (2011) · cites this paper as Zalfa et al., 2003
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Verkerk, 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 · see citing works
Self-citationInstability 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 · see citing works
Lyford, G. L., Yamagata, K., Kaufmann, W. E., Barnes, C. A., Sanders, L. K., Copeland, N. G., Gilbert, D. J., Jenkins, N. A., Lanahan, A. A., & Worley, P. F. (1995) · Neuron · doi:10.1016/0896-6273(95)90299-6 · Cited by 1,212 · see citing works
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 · see citing works
Fmr1 knockout mice: A model to study fragile X mental retardation
THEDUTCHBELGIANFRAGILEXCONSOR (1994) · Cell · doi:10.1016/0092-8674(94)90569-x · Cited by 1,075 · see citing works
Abnormal dendritic spines in fragile X knockout mice: Maturation and pruning deficits
Comery, T. A., Harris, J. B., Willems, P. J., Oostra, B. A., Irwin, S. A., Weiler, I. J., & Greenough, W. T. (1997) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.94.10.5401 · Cited by 1,046 · see citing works
Self-citationFragile 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
FMR1 Protein: Conserved RNP Family Domains and Selective RNA Binding
Ashley, C. T., Wilkinson, K. D., Reines, D., & Warren, S. T. (1993) · Science · doi:10.1126/science.7692601 · Cited by 741 · see citing works
Protein synthesis at synaptic sites on dendrites.
Steward, O., & Schuman, E. (2001) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.24.1.299 · Cited by 731 · see citing works
Somatodendritic expression of an immediate early gene is regulated by synaptic activity.
Link, W., Konietzko, U., Kauselmann, G., Krug, M., Schwanke, B., Frey, U., & Kuhl, D. (1995) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.92.12.5734 · Cited by 706 · see citing works
DNA methylation represses <i>FMR-1</i> transcription in fragile X syndrome
Sutcliffe, J. S., Nelson, D. L., Zhang, F., Pieretti, M., Caskey, C. T., Saxe, D., & Warren, S. T. (1992) · Human Molecular Genetics · doi:10.1093/hmg/1.6.397 · Cited by 680 · see citing works
Weiler, I. J., Irwin, S. A., Klintsova, A. Y., Spencer, C. M., Brazelton, A. D., Miyashiro, K., Comery, T. A., Patel, B., Eberwine, J., & Greenough, W. T. (1997) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.94.10.5395 · Cited by 644 · see citing works
Zhang, Y., Bailey, A. M., Matthies, H. J. G., Renden, R., Smith, M. A., Speese, S. D., Rubin, G. M., & Broadie, K. S. (2001) · Cell · doi:10.1016/s0092-8674(01)00589-x · Cited by 641 · see citing works
A point mutation in the FMR-1 gene associated with fragile X mental retardation
Boulle, K. D., Verkerk, A. J. M. H., Reyniers, E., Vits, L., Hendrickx, J., Roy, B. V., Bos, F. V. D., de Graaff, E., Oostra, B. A., & Willems, P. J. (1993) · Nature Genetics · doi:10.1038/ng0193-31 · Cited by 624 · see citing works
Self-citationEvidence 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
Abnormal Development of Dendritic Spines in<i>FMR1</i>Knock-Out Mice
Nimchinsky, E. A., Oberlander, A. M., & Svoboda, K. (2001) · The Journal of Neuroscience · doi:10.1523/jneurosci.21-14-05139.2001 · Cited by 560 · see citing works
Selective localization of messenger RNA for cytoskeletal protein MAP2 in dendrites
Garner, C. C., Tucker, R. P., & Matus, A. I. (1988) · Nature · doi:10.1038/336674a0 · Cited by 558 · see citing works
Feng, Y., Gutekunst, C.-A., Eberhart, D. E., Yi, H., Warren, S. T., & Hersch, S. M. (1997) · The Journal of Neuroscience · doi:10.1523/jneurosci.17-05-01539.1997 · Cited by 532 · see citing works
Analysis 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
A Decade of Molecular Studies of Fragile X Syndrome
O'Donnell, W. T., & Warren, S. T. (2002) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.25.112701.142909 · 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
The fragile X mental retardation protein inhibits translation via interacting with mRNA
Li, Z. (2001) · Nucleic Acids Research · doi:10.1093/nar/29.11.2276 · Cited by 448 · see citing works
Siomi, H., Choi, M., Siomi, M. C., Nussbaum, R. L., & Dreyfuss, G. (1994) · Cell · doi:10.1016/0092-8674(94)90232-1 · Cited by 446 · see citing works
Eberhart, D. (1996) · Human Molecular Genetics · doi:10.1093/hmg/5.8.1083 · Cited by 390 · see citing works
[20] Computer prediction of RNA structure
Zuker, M. (1989) · Methods in Enzymology · doi:10.1016/0076-6879(89)80106-5 · Cited by 388 · see citing works
Mazrouï, R. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.24.3007 · Cited by 369 · see citing works
Bagni, C., Mannucci, L., Dotti, C. G., & Amaldi, F. (2000) · Journal of Neuroscience · doi:10.1523/jneurosci.20-10-j0004.2000 · Cited by 357 · see citing works
Self-citationSchenck, A., Bardoni, B., Moro, A., Bagni, C., & Mandel, J.-L. (2001) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.151231598 · Cited by 356 · see citing works
Self-citationThe fragile X mental retardation syndrome protein interacts with novel homologs FXR1 and FXR2.
Zhang, Y., O'Connor, J. P., Siomi, M. C., Srinivasan, S., Dutra, A., Nussbaum, R. L., & Dreyfuss, G. (1995) · The EMBO Journal · doi:10.1002/j.1460-2075.1995.tb00220.x · Cited by 327 · see citing works
Synaptic regulation of protein synthesis and the fragile X protein
Greenough, W. T., Klintsova, A. Y., Irwin, S. A., Galvez, R., Bates, K. E., & Weiler, I. J. (2001) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.141145998 · Cited by 317 · see citing works
On "genomenclature": a comprehensive (and respectful) taxonomy for pseudogenes and other "junk DNA".
Brosius, J., & Gould, S. J. (1992) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.89.22.10706 · Cited by 316 · see citing works
Siomi, M. C., Zhang, Y., Siomi, H., & Dreyfuss, G. (1996) · Molecular and Cellular Biology · doi:10.1128/mcb.16.7.3825 · Cited by 271 · see citing works
Local protein synthesis and its role in synapse-specific plasticity
Martin, K. C., Barad, M., & Kandel, E. R. (2000) · Current Opinion in Neurobiology · doi:10.1016/s0959-4388(00)00128-8 · Cited by 258 · see citing works
The fragile X mental retardation protein is associated with ribosomes
Khandjian, E. W., Corbin, F., Woerly, S., & Rousseau, F. (1996) · Nature Genetics · doi:10.1038/ng0196-91 · Cited by 251 · see citing works
Corbin, F. (1997) · Human Molecular Genetics · doi:10.1093/hmg/6.9.1465 · Cited by 235 · see citing works
Dendritic BC1 RNA: Functional Role in Regulation of Translation Initiation
Wang, H., Iacoangeli, A., Popp, S., Muslimov, I., Imataka, H., Sonenberg, N., Lomakin, I., & Tiedge, H. (2002) · Journal of Neuroscience · doi:10.1523/jneurosci.22-23-10232.2002 · Cited by 232 · see citing works
CaM-kinases: modulators of synaptic plasticity
Soderling, T. R. (2000) · Current Opinion in Neurobiology · doi:10.1016/s0959-4388(00)00090-8 · Cited by 231 · see citing works
Dendritic location of neural BC1 RNA.
Tiedge, H., Fremeau, R. T., Weinstock, P. H., Arancio, O., & Brosius, J. (1991) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.88.6.2093 · Cited by 231 · see citing works
Primary structure, neural-specific expression, and dendritic location of human BC200 RNA
Tiedge, H., Chen, W., & Brosius, J. (1993) · Journal of Neuroscience · doi:10.1523/jneurosci.13-06-02382.1993 · Cited by 225 · see citing works
Fragile X syndrome without CCG amplification has an FMR1 deletion
Gedeon, Á. K., Baker, E., Robinson, H., Partington, M. W., Gross, B., Manca, A., Korn, B., Poustka, A., Yu, S., Sutherland, G. R., et al. (1992) · Nature Genetics · doi:10.1038/ng0892-341 · Cited by 220 · see citing works
Differential expression of FMR1, FXR1 and FXR2 proteins in human brain and testis
Tamanini, F. (1997) · Human Molecular Genetics · doi:10.1093/hmg/6.8.1315 · Cited by 198 · see citing works
Brown, V., Small, K. M., Lakkis, L., Feng, Y., Gunter, C., Wilkinson, K. D., & Warren, S. T. (1998) · Journal of Biological Chemistry · doi:10.1074/jbc.273.25.15521 · Cited by 192 · see citing works
Ceman, S., Brown, V., & Warren, S. T. (1999) · Molecular and Cellular Biology · doi:10.1128/mcb.19.12.7925 · Cited by 181 · see citing works
Wöhrle, D., Kotzot, D., Hirst, M. C., Manca, A., Korn, B., Schmidt, A., Barbi, G., Rott, H. D., Poustka, A., & Davies, K. E. (1992) · Europe PMC (PubMed Central) · Cited by 172 · see citing works
From factors to mechanisms: translation and translational control in eukaryotes
Preiß, T., & Hentze, M. W. (1999) · Current Opinion in Genetics & Development · doi:10.1016/s0959-437x(99)00005-2 · Cited by 154 · see citing works
RNA Transport in Dendrites: Acis-Acting Targeting Element Is Contained within Neuronal BC1 RNA
Muslimov, I. A., Santi, E., Homel, P., Perini, S., Higgins, D. M., & Tiedge, H. (1997) · Journal of Neuroscience · doi:10.1523/jneurosci.17-12-04722.1997 · Cited by 150 · see citing works
BC200 RNA: a neural RNA polymerase III product encoded by a monomeric Alu element.
Martignetti, J. A., & Brosius, J. (1993) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.90.24.11563 · Cited by 149 · see citing works
FMRP is associated to the ribosomes via RNA
Tamanini, F. (1996) · Human Molecular Genetics · doi:10.1093/hmg/5.6.809 · Cited by 137 · see citing works
Bardoni, B., Schenck, A., & Louis Mandel, J. (1999) · Human Molecular Genetics · doi:10.1093/hmg/8.13.2557 · Cited by 136 · see citing works
LTP, Memory and Structural Plasticity
Müller, D., Nikonenko, I., Jourdain, P., & Alberi, S. (2002) · Current Molecular Medicine · doi:10.2174/1566524023362041 · Cited by 136 · see citing works
Down syndrome consequent to a cryptic maternal 12p;21q chromosome translocation
Scott, J. A., Wenger, S. L., Steele, M. W., & Chakravarti, A. (1995) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320560115 · Cited by 130 · see citing works
Bardoni, B., & Mandel, J. (2002) · Current Opinion in Genetics & Development · doi:10.1016/s0959-437x(02)00300-3 · Cited by 129 · see citing works
Meljer, H., de Graaff, E., Merckx, D. M. L., Jongbloed, R. J. E., de Die-Smulders, C. E. M., Engelen, J. J. M., Fryns, J., Curfs, P. M. G., & Oostra, B. A. (1994) · Human Molecular Genetics · doi:10.1093/hmg/3.4.615 · Cited by 129 · see citing works
Self-citationAdinolfi, S., Bagni, C., Musco, G., Gibson, T. J., Mazzarella, L., & Pastore, A. (1999) · RNA · doi:10.1017/s1355838299990647 · Cited by 96 · see citing works
Self-citationRNAs That Interact with the Fragile X Syndrome RNA Binding Protein FMRP
Sung, Y. J., Conti, J., Currie, J. R., Brown, W. T., & Denman, R. B. (2000) · Biochemical and Biophysical Research Communications · doi:10.1006/bbrc.2000.3405 · Cited by 82 · see citing works
Identification of Mouse YB1/p50 as a Component of the FMRP-Associated mRNP Particle
Ceman, S., Nelson, R., & Warren, S. T. (2000) · Biochemical and Biophysical Research Communications · doi:10.1006/bbrc.2000.4035 · Cited by 81 · see citing works
Rozhdestvensky, T. S., Kopylov, A. M., Brosius, J., & Hüttenhofer, A. (2001) · RNA · doi:10.1017/s1355838201002485 · Cited by 79 · see citing works
Selective translation of mRNAs at synapses
Richter, J. D. (2002) · Current Opinion in Neurobiology · doi:10.1016/s0959-4388(02)00318-5 · Cited by 68 · see citing works
Identification and Characterization of BC1 RNP Particles
Cheng, J., Tiedge, H., & Brosius, J. (1996) · DNA and Cell Biology · doi:10.1089/dna.1996.15.549 · Cited by 50 · see citing works
Rare variants in the promoter of the fragile X syndrome gene (FMR1)
Milà, M., Castellvı́-Bel, S., Sánchez, A., Barceló, A., Bádenas, C., Mallolas, J., & Estivill, X. (2000) · Molecular and Cellular Probes · doi:10.1006/mcpr.2000.0293 · Cited by 25 · see citing works
Gordon‐Weeks, P. R. (1988) · Trends in Neurosciences · doi:10.1016/0166-2236(88)90054-9 · Cited by 24 · see citing works
Cheng, J., Tiedge, H., & Brosius, J. (1997) · Neuroscience Letters · doi:10.1016/s0304-3940(97)13471-1 · Cited by 23 · see citing works
Functions of the fragile X protein
Oostra, B. A. (2002) · Trends in Molecular Medicine · doi:10.1016/s1471-4914(02)02271-2 · Cited by 9 · see citing works
Self-citation
Flagged first, then by citations
2 of the 65 references couldn’t be matched to a known paper.
Cited by
Sorted by citations
Evolution and Functions of Long Noncoding RNAs
Ponting, C. P., Oliver, P. L., & Reik, W. (2009) · Cell · doi:10.1016/j.cell.2009.02.006 · Cited by 4,917 · see citing works
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
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
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
ERK signalling: A master regulator of cell behaviour, life and fate
Lavoie, H., Gagnon, J., & Therrien, M. (2020) · Nature Reviews Molecular Cell Biology · doi:10.1038/s41580-020-0255-7 · Cited by 1,185 · see citing works
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 · see citing works
Pten Regulates Neuronal Arborization and Social Interaction in Mice
Kwon, C.-H., Luikart, B. W., Powell, C. M., Zhou, J., Matheny, S. A., Zhang, W., Li, Y., Baker, S. J., & Parada, L. F. (2006) · Neuron · doi:10.1016/j.neuron.2006.03.023 · Cited by 970 · see citing works
Translational control of long-lasting synaptic plasticity and memory
Costa-Mattioli, M., Sossin, W. S., Klann, E., & Sonenberg, N. (2009) · Neuron · doi:10.1016/j.neuron.2008.10.055 · Cited by 944 · see citing works
Synaptic plasticity in the anterior cingulate cortex in acute and chronic pain
Bliss, T., Collingridge, G. L., Kaang, B., & Zhuo, M. (2016) · Nature reviews. Neuroscience · doi:10.1038/nrn.2016.68 · Cited by 781 · see citing works
Arc/Arg3.1 Mediates Homeostatic Synaptic Scaling of AMPA Receptors
Shepherd, J. D., Rumbaugh, G., Wu, J., Chowdhury, S., Plath, N., Kuhl, D., Huganir, R. L., & Worley, P. F. (2006) · Neuron · doi:10.1016/j.neuron.2006.08.034 · Cited by 779 · see citing works
Panzitt, K., Tschernatsch, M. M. O., Guelly, C., Moustafa, T., Stradner, M. H., Strohmaier, H. M., Buck, C. R., Denk, H., Schroeder, R., Trauner, M. A., et al. (2006) · Gastroenterology · doi:10.1053/j.gastro.2006.08.026 · Cited by 771 · see citing works
Han, T. W., Kato, M., Xie, S., Wu, L. C., Mirzaei, H., Pei, J., Chen, M., Xie, Y., Allen, J. D., Xiao, G., et al. (2012) · Cell · doi:10.1016/j.cell.2012.04.016 · Cited by 758 · see citing works
Activity-dependent neuronal signalling and autism spectrum disorder
Ebert, D. H., & Greenberg, M. E. (2013) · Nature · doi:10.1038/nature11860 · Cited by 666 · see citing works
Normal huntingtin function: an alternative approach to Huntington's disease
Cattaneo, E. F., Zuccato, C., & Tartari, M. (2005) · Nature reviews. Neuroscience · doi:10.1038/nrn1806 · Cited by 655 · see citing works
Jacquemont, S., Curie, A., des Portes, V., Torrioli, M. G., Berry-Kravis, E., Hagerman, R. J., Ramos, F. J., Cornish, K., He, Y., Paulding, C., et al. (2011) · Science Translational Medicine · doi:10.1126/scitranslmed.3001708 · Cited by 609 · see citing works
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 · see citing works
Self-citationJin, P., Zarnescu, D. C., Ceman, S., Nakamoto, M., Mowrey, J., Jongens, T. A., Nelson, D. L., Moses, K., & Warren, S. T. (2004) · Nature Neuroscience · doi:10.1038/nn1174 · Cited by 604 · see citing works
Translational Regulatory Mechanisms in Persistent Forms of Synaptic Plasticity
Kelleher, R. J., Govindarajan, A., & Tonegawa, S. (2004) · Neuron · doi:10.1016/j.neuron.2004.09.013 · Cited by 592 · see citing works
Dendritic mRNA: transport, translation and function
Bramham, C. R., & Wells, D. G. (2007) · Nature reviews. Neuroscience · doi:10.1038/nrn2150 · Cited by 574 · see citing works
Eiring, A. M., Harb, J. G., Neviani, P., Garton, C., Oaks, J. J., Spizzo, R., Liu, S., Schwind, S., Santhanam, R., Hickey, C. J., et al. (2010) · Cell · doi:10.1016/j.cell.2010.01.007 · Cited by 560 · see citing works
The expanding RNA polymerase III transcriptome
Dieci, G., Fiorino, G., Castelnuovo, M., Teichmann, M., & Pagano, A. (2007) · Trends in Genetics · doi:10.1016/j.tig.2007.09.001 · Cited by 536 · see citing works
Molecular 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
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
Park, S., Park, J. M., Kim, S., Kim, J.-A., Shepherd, J. D., Smith-Hicks, C. L., Chowdhury, S., Kaufmann, W., Kuhl, D., Ryazanov, A. G., et al. (2008) · Neuron · doi:10.1016/j.neuron.2008.05.023 · Cited by 516 · see citing works
The Intense World Theory – A Unifying Theory of the Neurobiology of Autism
Markram, H., & Markram, H. (2010) · Frontiers in Human Neuroscience · doi:10.3389/fnhum.2010.00224 · Cited by 522 · see citing works
Dai, M., Zeng, S. X., Jin, Y., Sun, X., David, L. L., & Lu, H. (2004) · Molecular and Cellular Biology · doi:10.1128/mcb.24.17.7654-7668.2004 · Cited by 495 · see citing works
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 · see citing works
Dictenberg, J. B., Swanger, S. A., Antar, L. N., Singer, R. H., & Bassell, G. J. (2008) · Developmental Cell · doi:10.1016/j.devcel.2008.04.003 · Cited by 511 · see citing works
From mRNP trafficking to spine dysmorphogenesis: the roots of fragile X syndrome
Bagni, C., & Greenough, W. T. (2005) · Nature Reviews Neuroscience · doi:10.1038/nrn1667 · Cited by 475 · see citing works
Self-citationWaung, M. W., Pfeiffer, B. E., Nosyreva, E. D., Ronesi, J. A., & Huber, K. M. (2008) · Neuron · doi:10.1016/j.neuron.2008.05.014 · Cited by 474 · see citing works
Hou, L., Antion, M. D., Hu, D., Spencer, C. M., Paylor, R., & Klann, E. (2006) · Neuron · doi:10.1016/j.neuron.2006.07.005 · Cited by 469 · see citing works
Dendritic Spines: The Locus of Structural and Functional Plasticity
Sala, C., & Segal, M. (2014) · Physiological Reviews · doi:10.1152/physrev.00012.2013 · Cited by 465 · see citing works
Bramham, C. R., Alme, M. N., Bittins, M., Kuipers, S. D., Nair, R. R., Pai, B., Panja, D., Schubert, M., Soule, J., Tiron, A., et al. (2010) · Experimental Brain Research · doi:10.1007/s00221-009-1959-2 · Cited by 452 · see citing works
Briggs, J., Wolvetang, E. J., Mattick, J. S., Rinn, J. L., & Barry, G. (2015) · Neuron · doi:10.1016/j.neuron.2015.09.045 · Cited by 443 · see citing works
The Aβ oligomer hypothesis for synapse failure and memory loss in Alzheimer’s disease
Ferreira, S. T., & Klein, W. L. (2011) · Neurobiology of Learning and Memory · doi:10.1016/j.nlm.2011.08.003 · Cited by 442 · see citing works
NONCODING RNAS IN THE MAMMALIAN CENTRAL NERVOUS SYSTEM
Cao, X., Yeo, G. W.-M., Muotri, A. R., Kuwabara, T., & Gage, F. H. (2006) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.29.051605.112839 · Cited by 432 · see citing works
Muddashetty, R. S., Kelić, S., Gross, C., Xu, M., & Bassell, G. J. (2007) · The Journal of Neuroscience · doi:10.1523/jneurosci.0937-07.2007 · Cited by 421 · see citing works
The Pathophysiology of Fragile X Syndrome
Penagarikano, O., Mulle, J. G., & Warren, S. T. (2007) · Annual Review of Genomics and Human Genetics · doi:10.1146/annurev.genom.8.080706.092249 · Cited by 420 · see citing works
The FMR1 premutation and reproduction
Wittenberger, M. D., Hagerman, R. J., Sherman, S. L., McConkie‐Rosell, A., Welt, C. K., Rebar, R. W., Corrigan, E. C., Simpson, J. L., & Nelson, L. M. (2006) · Fertility and Sterility · doi:10.1016/j.fertnstert.2006.09.004 · Cited by 412 · see citing works
Eukaryotic regulatory RNAs: an answer to the ‘genome complexity’ conundrumPreprint
Prasanth, K. V., & Spector, D. L. (2007) · Genes & Development · doi:10.1101/gad.1484207 · Cited by 398 · see citing works
MicroRNAs in Disease and Potential Therapeutic Applications
Soifer, H. S., Rossi, J. J., & Sætrom, P. (2007) · Molecular Therapy · doi:10.1038/sj.mt.6300311 · Cited by 394 · see citing works
Antar, L. N., Afroz, R., Dictenberg, J. B., Carroll, R. C., & Bassell, G. J. (2004) · The Journal of Neuroscience · doi:10.1523/jneurosci.0099-04.2004 · Cited by 360 · see citing works
Arc in synaptic plasticity: from gene to behavior
Korb, E., & Finkbeiner, S. (2011) · Trends in Neurosciences · doi:10.1016/j.tins.2011.08.007 · Cited by 388 · see citing works
Osterweil, E. K., Krueger, D. D., Reinhold, K., & Bear, M. F. (2010) · The Journal of Neuroscience · doi:10.1523/jneurosci.3888-10.2010 · Cited by 384 · see citing works
Compartmentalized Synthesis and Degradation of Proteins in Neurons
Steward, O., & Schuman, E. M. (2003) · Neuron · doi:10.1016/s0896-6273(03)00635-4 · Cited by 377 · see citing works
AN ABUNDANCE OF RNA REGULATORS
Storz, G., Altuvia, S., & Wassarman, K. M. (2005) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.74.082803.133136 · Cited by 370 · see citing works
Ramı́rez-Amaya, V., Vazdarjanova, A. I., Mikhael, D. M., Rosi, S., Worley, P. F., & Barnes, C. A. (2005) · Journal of Neuroscience · doi:10.1523/jneurosci.4342-04.2005 · Cited by 364 · see citing works
A 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
Self-citationNon-coding RNAs: New players in eukaryotic biology
Costa, F. F. (2005) · Gene · doi:10.1016/j.gene.2005.06.019 · Cited by 353 · see citing works
Making synaptic plasticity and memory last: mechanisms of translational regulationPreprint
Richter, J. D., & Klann, E. (2009) · Genes & Development · doi:10.1101/gad.1735809 · Cited by 345 · see citing works
TDP‐43, the signature protein of FTLD‐U, is a neuronal activity‐responsive factor
Wang, I., Wu, L., Chang, H., & Shen, C. ‐. J. (2007) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2007.05190.x · Cited by 334 · see citing works
Mazumder, B., Sampath, P., Seshadri, V., Maitra, R. K., DiCorleto, P. E., & Fox, P. L. (2003) · Cell · doi:10.1016/s0092-8674(03)00773-6 · Cited by 334 · see citing works
Phosphorylation influences the translation state of FMRP-associated polyribosomes
Ceman, S., O'Donnell, W. T., Reed, M., Patton, S., Pohl, J., & Warren, S. T. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg350 · Cited by 327 · see citing works
A synaptic perspective of fragile X syndrome and autism spectrum Disorders.
Bagni, C., & Zukin, R. S. (2019) · Neuron · doi:10.1016/j.neuron.2019.02.041 · Cited by 326 · see citing works
Self-citationLu, 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
Fragile X syndrome: causes, diagnosis, mechanisms, and therapeutics
Bagni, C., Tassone, F., Neri, G., & Hagerman, R. (2012) · Journal of Clinical Investigation · doi:10.1172/jci63141 · Cited by 321 · see citing works
Self-citationRho GTPases, dendritic structure, and mental retardation
Newey, S. E., Velamoor, V., Govek, E., & Van Aelst, L. (2005) · Journal of Neurobiology · doi:10.1002/neu.20153 · Cited by 351 · see citing works
Decreased expression of the GABAA receptor in fragile X syndrome
D'Hulst, C., De Geest, N., Reeve, S. P., Van Dam, D., De Deyn, P. P., Hassan, B. A., & Kooy, R. F. (2006) · Brain Research · doi:10.1016/j.brainres.2006.08.115 · Cited by 318 · see citing works
Schenck, A., Bardoni, B., Langmann, C., Harden, N., Mandel, J.-L., & Giangrande, A. (2003) · Neuron · doi:10.1016/s0896-6273(03)00354-4 · Cited by 316 · see citing works
Remote Control of Gene Function by Local Translation
Jung, H., Gkogkas, C. G., Sonenberg, N., & Holt, C. E. (2014) · Cell · doi:10.1016/j.cell.2014.03.005 · Cited by 302 · see citing works
Restivo, L., Ferrari, F., Passino, E., Sgobio, C., Bock, J., Oostra, B. A., Bagni, C., & Ammassari-Teule, M. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0504984102 · Cited by 299 · see citing works
Self-citationDe Rubeis, S., Pasciuto, E., Li, K. W., Fernández, E., Di Marino, D., Buzzi, A., Ostroff, L. E., Klann, E., Zwartkruis, F. J. T., Komiyama, N. H., et al. (2013) · Neuron · doi:10.1016/j.neuron.2013.06.039 · Cited by 296 · see citing works
Self-citationFMRP Mediates mGluR5-Dependent Translation of Amyloid Precursor Protein
Westmark, C. J., & Malter, J. S. (2007) · PLoS Biology · doi:10.1371/journal.pbio.0050052 · Cited by 292 · see citing works
Nosyreva, E. D., & Huber, K. M. (2006) · Journal of Neurophysiology · doi:10.1152/jn.01316.2005 · Cited by 292 · 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
The generation of a conditional Fmr1 knock out mouse model to study Fmrp function in vivo
Mientjes, E., Nieuwenhuizen, I. M., Kirkpatrick, L., Zu, T., Hoogeveen‐Westerveld, M., Severijnen, L., Rife, M. F., Willemsen, R., Nelson, D. L., & Oostra, B. A. (2005) · Neurobiology of Disease · doi:10.1016/j.nbd.2005.08.019 · Cited by 285 · see citing works
Self-citationCampalans, A., Kondorosi, Á., & Crespi, M. D. (2004) · The Plant Cell · doi:10.1105/tpc.019406 · Cited by 283 · see citing works
Fragile-X syndrome and fragile X-associated tremor/ataxia syndrome: two faces of FMR1
Jacquemont, S., Hagerman, R. J., Hagerman, P. J., & Leehey, M. A. (2006) · The Lancet Neurology · doi:10.1016/s1474-4422(06)70676-7 · Cited by 277 · see citing works
Nishimura, Y., Martin, C. L., Vazquez-Lopez, A., Spence, S. J., Alvarez-Retuerto, A. I., Sigman, M., Steindler, C., Pellegrini, S., Schanen, N. C., Warren, S. T., et al. (2007) · Human Molecular Genetics · doi:10.1093/hmg/ddm116 · Cited by 276 · see citing works
Dysregulation and restoration of translational homeostasis in fragile X syndrome
Richter, J. D., Bassell, G. J., & Klann, E. (2015) · Nature Reviews Neuroscience · doi:10.1038/nrn4001 · Cited by 275 · 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
Disruption 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
Berry-Kravis, E., Des Portes, V., Hagerman, R., Jacquemont, S., Charles, P., Visootsak, J., Brinkman, M., Rerat, K., Koumaras, B., Zhu, L., et al. (2016) · Science Translational Medicine · doi:10.1126/scitranslmed.aab4109 · Cited by 270 · see citing works
From junk to master regulators of invasion: lncRNA functions in migration, EMT and metastasis
Dhamija, S., & Diederichs, S. (2016) · International Journal of Cancer · doi:10.1002/ijc.30039 · Cited by 268 · see citing works
Signalling pathways in autism spectrum disorder: mechanisms and therapeutic implications
Jiang, C.-C., Lin, L., Long, S., Ke, X., Fukunaga, K., Lu, Y., & Han, F. (2022) · Signal Transduction and Targeted Therapy · doi:10.1038/s41392-022-01081-0 · Cited by 272 · see citing works
Molecular and Comparative Genetics of Mental Retardation
Inlow, J. K., & Restifo, L. L. (2004) · Genetics · doi:10.1534/genetics.166.2.835 · Cited by 262 · see citing works
Fragile X mental retardation protein controls gating of the sodium-activated potassium channel Slack
Brown, M. R., Kronengold, J., Gazula, V., Chen, Y., Strumbos, J. G., Sigworth, F. J., Navaratnam, D. S., & Kaczmarek, L. K. (2010) · Nature Neuroscience · doi:10.1038/nn.2563 · Cited by 261 · see citing works
Antar, L. N., Li, C., Zhang, H., Carroll, R. C., & Bassell, G. J. (2006) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2006.02.001 · Cited by 259 · see citing works
McIntyre, C. K., Miyashita, T., Setlow, B., Marjon, K. D., Steward, O., Guzowski, J. F., & McGaugh, J. L. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0504436102 · Cited by 252 · see citing works
Disrupted Homer scaffolds mediate abnormal mGluR5 function in a mouse model of fragile X syndrome
Ronesi, J. A., Collins, K. A., Hays, S. A., Tsai, N.-P., Guo, W., Birnbaum, S. G., Hu, J.-H., Worley, P. F., Gibson, J. R., & Huber, K. M. (2012) · Nature Neuroscience · doi:10.1038/nn.3033 · Cited by 251 · see citing works
Gong, R., Park, C. S., Abbassi, N. R., & Tang, S.-J. (2006) · Journal of Biological Chemistry · doi:10.1074/jbc.m512524200 · Cited by 249 · see citing works
Fragile X Mental Retardation Protein Regulates Translation by Binding Directly to the Ribosome
Chen, E., Sharma, M. R., Shi, X., Agrawal, R. K., & Joseph, S. (2014) · Molecular Cell · doi:10.1016/j.molcel.2014.03.023 · Cited by 240 · see citing works
Antar, L. N., Dictenberg, J. B., Plociniak, M., Afroz, R., & Bassell, G. J. (2005) · Genes Brain & Behavior · doi:10.1111/j.1601-183x.2005.00128.x · Cited by 246 · see citing works
Fragile X mental retardation protein and synaptic plasticity
Sidorov, M. S., Auerbach, B. D., & Bear, M. F. (2013) · Molecular Brain · doi:10.1186/1756-6606-6-15 · Cited by 243 · 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
Brain-Derived Neurotrophic Factor Rescues Synaptic Plasticity in a Mouse Model of Fragile X Syndrome
Lauterborn, J. C., Rex, C. S., Kramár, E. A., Chen, L. Y., Pandyarajan, V., LYNCH, G. S., & Gall, C. M. (2007) · Journal of Neuroscience · doi:10.1523/jneurosci.2624-07.2007 · Cited by 241 · see citing works
The Function of Activity-Regulated Genes in the Nervous System
Loebrich, S., & Nedivi, E. (2009) · Physiological Reviews · doi:10.1152/physrev.00013.2009 · Cited by 235 · see citing works
Lee, A., Li, W., Xu, K., Bogert, B. A., Su, K., & Gao, F. (2003) · Development · doi:10.1242/dev.00792 · Cited by 226 · see citing works
RNA TRANSPORT AND LOCAL CONTROL OF TRANSLATION
Kindler, S., Wang, H., Richter, D., & Tiedge, H. (2005) · Annual Review of Cell and Developmental Biology · doi:10.1146/annurev.cellbio.21.122303.120653 · Cited by 215 · see citing works
The State of Synapses in Fragile X Syndrome
Pfeiffer, B. E., & Huber, K. M. (2009) · The Neuroscientist · doi:10.1177/1073858409333075 · Cited by 214 · 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
Gatto (2010) · Frontiers in Synaptic Neuroscience · doi:10.3389/fnsyn.2010.00004 · Cited by 204 · see citing works
ElevatedFMR1mRNA in premutation carriers is due to increased transcription
Tassone, F., Beilina, A. G., Carosi, C., Albertosi, S., Bagni, C., Li, L., Glover-Cutter, K. M., Bentley, D. L., & Hagerman, P. J. (2007) · RNA · doi:10.1261/rna.280807 · Cited by 199 · see citing works
Self-citationThe G-quartet containing FMRP binding site in FMR1 mRNA is a potent exonic splicing enhancer
Didiot, M.-C., Tian, Z., Schaeffer, C., Subramanian, M., Mandel, J., & Moine, H. (2008) · Nucleic Acids Research · doi:10.1093/nar/gkn472 · Cited by 196 · see citing works
Non-coding RNA Generated following Lariat Debranching Mediates Targeting of AID to DNA
Zheng, S., Vuong, B. Q., Vaidyanathan, B., Lin, J., Huang, F., & Chaudhuri, J. (2015) · Cell · doi:10.1016/j.cell.2015.03.020 · Cited by 194 · see citing works
Local translational control in dendrites and its role in long‐term synaptic plasticity
Sutton, M. A., & Schuman, E. M. (2005) · Journal of Neurobiology · doi:10.1002/neu.20152 · Cited by 188 · see citing works
Meredith, R. M., Holmgren, C. D., Weidum, M., Burnashev, N., & Mansvelder, H. D. (2007) · Neuron · doi:10.1016/j.neuron.2007.04.028 · Cited by 190 · see citing works
Visualization of RNA–protein interactions in living cells: FMRP and IMP1 interact on mRNAs
Rackham, O., & Brown, C. M. (2004) · The EMBO Journal · doi:10.1038/sj.emboj.7600341 · Cited by 189 · see citing works
Pfeiffer, B. E., & Huber, K. M. (2007) · The Journal of Neuroscience · doi:10.1523/jneurosci.0054-07.2007 · Cited by 154 · see citing works
Targeting Homeostatic Synaptic Plasticity for Treatment of Mood Disorders
Kavalali, E. T., & Monteggia, L. M. (2020) · Neuron · doi:10.1016/j.neuron.2020.05.015 · Cited by 179 · see citing works
- Authors
- Zalfa, F., Giorgi, M., Primerano, B., Moro, A., Di Penta, A., Reis, S., Oostra, B., & Bagni, C.
- Affiliations
- University of Rome Tor Vergata (IT)
- Erasmus MC (NL)
- Corresponding author
- Bagni, Claudia
- Venue
- Cell
- Publisher
- Elsevier BV
- Published
- 2003-02
- Volume
- 112
- Issue
- 3
- Pages
- 317-327
- ISSN
- 0092-8674 (ISSN-L)
- OpenAlex
- W2002364516
- Cited by
- 676
- References
- 65
- Citations by year
- 2026: 4 · 2025: 13 · 2024: 14 · 2023: 13 · 2022: 9 · 2021: 21 · 2020: 19 · 2019: 27 · 2018: 28 · 2017: 36 · 2016: 19 · 2015: 33 · 2014: 39 · 2013: 31 · 2012: 36
- FWCI
- 26.25
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Cell (publishedVersion)
- PubMed (publishedVersion)
- EUR Research Repository (Erasmus University Rotterdam) (publishedVersion)
- Lirias (submittedVersion, other-oa)
- EUR Research Repository (Erasmus University Rotterdam) (submittedVersion)
- Cineca Institutional Research Information System (Tor Vergata University) (submittedVersion)
- Funders
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (5R01 HD 38038, R01HD038038)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA Research and Splicing
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA and protein synthesis mechanisms
- Keywords
- fragile X syndrome, FMRP, BC1 RNA, protein synthesis inhibition, synaptic dysfunction, intellectual disability, RNA-binding proteins
- MeSH terms
- Animals, Base Sequence, Brain, Dendrites, Fragile X Syndrome, Humans, Molecular Sequence Data, Nerve Tissue Proteins, Synaptic Transmission, Protein Binding, Ribonucleoproteins, RNA, Messenger, Synapses, Protein Biosynthesis, Tumor Cells, Cultured, Molecular Structure, Genes, Suppressor, RNA-Binding Proteins, Mice, Knockout, RNA, Small Cytoplasmic, Macromolecular Substances, Mice, Fragile X Messenger Ribonucleoprotein 1
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