The translation of translational control by FMRP: therapeutic targets for FXS
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De novo protein synthesis is necessary for long-lasting modifications in synaptic strength and dendritic spine dynamics that underlie cognition. Fragile X syndrome (FXS), characterized by intellectual disability and autistic behaviors, holds promise for revealing the molecular basis for these long-term changes in neuronal function. Loss of function of the fragile X mental retardation protein (FMRP) results in defects in synaptic plasticity and cognition in many models of the disease. FMRP is a polyribosome-associated RNA-binding protein that regulates the synthesis of a set of plasticity-reated proteins by stalling ribosomal translocation on target mRNAs. The recent identification of mRNA targets of FMRP and its upstream regulators, and the use of small molecules to stall ribosomes in the absence of FMRP, have the potential to be translated into new therapeutic avenues for the treatment of FXS.
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Enhanced mTOR signaling and cap-dependent translation initiation complex are found also in mouse models of human Fragile X Syndrome, characterized by the lack of FMRP.
“Enhanced mTOR signaling and cap-dependent translation initiation complex are found also in mouse models of human Fragile X Syndrome, characterized by the lack of FMRP.85, 86, 87”
From Maternal immune activation dysregulation of the fetal brain transcriptome and relevance to the pathophysiology of autism spectrum disorder (2018) · cites this paper as [87]
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Requirement for Protein Synthesis at Developing Synapses
Sebeo, J., Hsiao, K., Bozdagi, O., Dumitriu, D., Ge, Y., Zhou, Q., & Benson, D. L. (2009) · Journal of Neuroscience · doi:10.1523/jneurosci.2613-09.2009 · Cited by 33 · see citing works
RetractedVerkerk, 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
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
Self-citationFunctional impact of global rare copy number variation in autism spectrum disorders
Pinto, D., Pagnamenta, A. T., Klei, L., Anney, R., Merico, D., Regan, R., Conroy, J., Magalhaes, T. R., Correia, C., Abrahams, B. S., et al. (2010) · Nature · doi:10.1038/nature09146 · Cited by 2,013 · 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
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
mTOR signaling: At the crossroads of plasticity, memory and disease
Hoeffer, C. A., & Klann, E. (2010) · Trends in Neurosciences · doi:10.1016/j.tins.2009.11.003 · Cited by 1,128 · see citing works
Self-citationAbnormal 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
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 1,013 · see citing works
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
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
Self-citationFMR1 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
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 · 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
The protein product of the fragile X gene, FMR1, has characteristics of an RNA-binding protein.
Siomi, H., Siomi, M. C., Nussbaum, R. L., & Dreyfuss, G. (1993) · Cell · doi:10.1016/0092-8674(93)90420-u · 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
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
The 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
Mutations causing syndromic autism define an axis of synaptic pathophysiology
Auerbach, B. D., Osterweil, E. K., & Bear, M. F. (2011) · Nature · doi:10.1038/nature10658 · Cited by 592 · 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
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
Dysregulation of mTOR signaling in fragile X syndrome
Sharma, A., Hoeffer, C. A., Takayasu, Y., Miyawaki, T., McBride, S. M., Klann, E., & Zukin, R. S. (2010) · The Journal of Neuroscience · doi:10.1523/jneurosci.3696-09.2010 · Cited by 557 · see citing works
Self-citationGibson, J. R., Bartley, A. F., Hays, S. A., & Huber, K. M. (2008) · Journal of Neurophysiology · doi:10.1152/jn.90752.2008 · Cited by 529 · 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
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
Chronic Pharmacological mGlu5 Inhibition Corrects Fragile X in Adult Mice
Michalon, A., Sidorov, M., Ballard, T. M., Ozmen, L., Spooren, W., Wettstein, J. G., Jaeschke, G., Bear, M. F., & Lindemann, L. (2012) · Neuron · doi:10.1016/j.neuron.2012.03.009 · Cited by 489 · 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
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
Self-citationBilousova, T. V., Dansie, L., Ngo, M., Aye, J., Charles, J. R., Ethell, D. W., & Ethell, I. M. (2009) · Journal of Medical Genetics · doi:10.1136/jmg.2008.061796 · Cited by 465 · 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
Minocycline and Neurodegenerative Diseases
Kim, H.-S., & Suh, Y.-H. (2009) · Behavioural Brain Research · doi:10.1016/j.bbr.2008.09.040 · Cited by 447 · see citing works
Özcan, U., Özcan, L., Yılmaz, E., Düvel, K., Şahin, M., Manning, B. D., & Hotamışlıgil, G. S. (2008) · Molecular Cell · doi:10.1016/j.molcel.2007.12.023 · Cited by 434 · see citing works
Biochemical mechanisms for translational regulation in synaptic plasticity
Klann, E., & Dever, T. E. (2004) · Nature reviews. Neuroscience · doi:10.1038/nrn1557 · Cited by 422 · see citing works
Self-citationA pilot open label, single dose trial of fenobam in adults with fragile X syndrome
Berry-Kravis, E., Hessl, D., Coffey, S., Hervey, C., Schneider, A., Yuhas, J., Hutchison, J., Snape, M., Tranfaglia, M., Nguyen, D. V., et al. (2009) · Journal of Medical Genetics · doi:10.1136/jmg.2008.063701 · Cited by 411 · see citing works
Tuberous sclerosis: a GAP at the crossroads of multiple signaling pathways
Kwiatkowski, D. J., & Manning, B. D. (2005) · Human Molecular Genetics · doi:10.1093/hmg/ddi260 · Cited by 395 · 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
HITS-CLIP: panoramic views of protein-RNA regulation in living cells
Darnell, R. B. (2010) · WIREs RNA · doi:10.1002/wrna.31 · Cited by 382 · see citing works
Exaggerated translation causes synaptic and behavioural aberrations associated with autism
Santini, E., Huynh, T. N., MacAskill, A. F., Carter, A. G., Pierre, P., Ruggero, D., Kaphzan, H., & Klann, E. (2012) · Nature · doi:10.1038/nature11782 · Cited by 376 · see citing works
Self-citationThe 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
Rho 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
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
Self-citationBerry-Kravis, E. M., Hessl, D., Rathmell, B., Zarevics, P., Cherubini, M., Walton-Bowen, K., Mu, Y., Nguyen, D. V., Gonzalez-Heydrich, J., Wang, P. P., et al. (2012) · Science Translational Medicine · doi:10.1126/scitranslmed.3004214 · Cited by 328 · see citing works
Delayed Stabilization of Dendritic Spines in Fragile X Mice
Cruz-Martin, A., Crespo, M., & Portera-Cailliau, C. (2010) · Journal of Neuroscience · doi:10.1523/jneurosci.0577-10.2010 · Cited by 321 · see citing works
Sequence-Specific RNA Binding by a Nova KH Domain
Lewis, H. A., Musunuru, K., Jensen, K. B., Edo, C., Chen, H., Darnell, R. B., & Burley, S. K. (2000) · Cell · doi:10.1016/s0092-8674(00)80668-6 · Cited by 319 · 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
Bhattacharya, A., Kaphzan, H., Alvarez-Dieppa, A. C., Murphy, J. P., Pierre, P., & Klann, E. (2012) · Neuron · doi:10.1016/j.neuron.2012.07.022 · Cited by 316 · see citing works
Self-citationNosyreva, 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
Self-citationFragile 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
Self-citationLi, J., Pelletier, M. R., Perez-Velazquez, J. L., & Carlen, P. L. (2002) · Molecular and Cellular Neuroscience · doi:10.1006/mcne.2001.1085 · Cited by 271 · see citing works
Qin, 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
SynGAP regulates synaptic strength and mitogen-activated protein kinases in cultured neurons
Rumbaugh, G., Adams, J. P., Kim, J. H., & Huganir, R. L. (2006) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0600084103 · Cited by 260 · 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
Identification of small molecules rescuing fragile X syndrome phenotypes in Drosophila
Chang, S., Bray, S. M., Li, Z., Zarnescu, D. C., He, C., Jin, P., & Warren, S. T. (2008) · Nature Chemical Biology · doi:10.1038/nchembio.78 · Cited by 254 · see citing works
Critical period plasticity is disrupted in the barrel cortex of FMR1 knockout mice
Harlow, E. G., Till, S. M., Russell, T. A., Wijetunge, L. S., Kind, P., & Contractor, A. (2010) · Neuron · doi:10.1016/j.neuron.2010.01.024 · 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
Henderson, C., Wijetunge, L., Kinoshita, M. N., Shumway, M., Hammond, R. S., Postma, F. R., Brynczka, C., Rush, R., Thomas, A., Paylor, R., et al. (2012) · Science Translational Medicine · doi:10.1126/scitranslmed.3004218 · Cited by 249 · see citing works
Ronesi, J. A., & Huber, K. M. (2008) · Journal of Neuroscience · doi:10.1523/jneurosci.5019-07.2008 · Cited by 244 · 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
Corbin, F. (1997) · Human Molecular Genetics · doi:10.1093/hmg/6.9.1465 · Cited by 235 · see citing works
Osterweil, E. K., Chuang, S.-C., Chubykin, A. A., Sidorov, M., Bianchi, R., Wong, R. K. S., & Bear, M. F. (2013) · Neuron · doi:10.1016/j.neuron.2012.01.034 · Cited by 230 · see citing works
Open-label add-on treatment trial of minocycline in fragile X syndrome.
Paribello, C., Tao, L., Folino, A., Berry-Kravis, E., Tranfaglia, M., Ethell, I. M., & Ethell, D. W. (2010) · BMC Neurology · doi:10.1186/1471-2377-10-91 · Cited by 223 · see citing works
Decreased GABAA receptor expression in the seizure-prone fragile X mouse
Idrissi, A. E., Ding, X., Scalia, J., Trenkner, E., Brown, W. T., & Dobkin, C. (2004) · Neuroscience Letters · doi:10.1016/j.neulet.2004.11.087 · Cited by 210 · see citing works
Excess protein synthesis in Drosophila Fragile X mutants impairs long-term memory
Bolduc, F. V., Bell, K., Cox, H., Broadie, K. S., & Tully, T. (2008) · Nature Neuroscience · doi:10.1038/nn.2175 · Cited by 210 · see citing works
Pan, F., Aldridge, G. M., Greenough, W. T., & Gan, W.-B. (2010) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1012496107 · Cited by 208 · see citing works
The FXG: a presynaptic fragile X granule expressed in a subset of developing brain circuits
Christie, S. B., Akins, M. R., Schwob, J. E., & Fallon, J. R. (2009) · The Journal of Neuroscience · doi:10.1523/jneurosci.3937-08.2009 · Cited by 208 · see citing works
Mines, M. A., Yuskaitis, C. J., King, M. K., Beurel, E., & Jope, R. S. (2010) · PLoS ONE · doi:10.1371/journal.pone.0009706 · Cited by 208 · see citing works
Altered mTOR signaling and enhanced CYFIP2 expression levels in subjects with fragile X syndrome
Hoeffer, C. A., Sanchez, E., Hagerman, R. J., Mu, Y., Nguyen, D. V., Wong, H., Whelan, A. M., Zukin, R. S., Klann, E., & Tassone, F. (2012) · Genes, Brain and Behavior · doi:10.1111/j.1601-183x.2012.00768.x · Cited by 198 · see citing works
Self-citationShanks, N. F., Savas, J. N., Maruo, T., Cais, O., Hirao, A., Oe, S., Ghosh, A., Noda, Y., Greger, I. H., Yates, J. R., et al. (2012) · Cell Reports · doi:10.1016/j.celrep.2012.05.004 · Cited by 182 · see citing works
Romanelli, A., Martin, K. A., Toker, A., & Blenis, J. (1999) · Molecular and Cellular Biology · doi:10.1128/mcb.19.4.2921 · Cited by 180 · 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
The role of protein synthesis during the labile phases of memory: Revisiting the skepticism
Alberini, C. M. (2007) · Neurobiology of Learning and Memory · doi:10.1016/j.nlm.2007.08.007 · Cited by 178 · see citing works
Guo, W., Murthy, A. C., Zhang, L., Johnson, E. B., Schaller, E., Allan, A. M., & Zhao, X. (2011) · Human Molecular Genetics · doi:10.1093/hmg/ddr501 · Cited by 159 · 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
Cognitive dysfunction and prefrontal synaptic abnormalities in a mouse model of fragile X syndrome
Krueger‐Burg, D., Osterweil, E. K., Chen, S. P., Tye, L. D., & Bear, M. F. (2011) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1013855108 · Cited by 153 · see citing works
Lee, H. Y., Ge, W., Huang, W. C., He, Y., Wang, G. X., Rowson-Baldwin, A., Smith, S. J., Jan, Y. N., & Jan, L. Y. (2011) · Neuron · doi:10.1016/j.neuron.2011.09.033 · Cited by 150 · see citing works
Deregulation of <i>EIF4E</i>: a novel mechanism for autism
Neves-Pereira, M., Müller, B., Massie, D., Williams, J. H. G., O’Brien, P. C. M., Hughes, A., Shen, S.-B., Clair, D. S., & Miedzybrodzka, Z. (2009) · Journal of Medical Genetics · doi:10.1136/jmg.2009.066852 · Cited by 142 · see citing works
Early developmental alterations in GABAergic protein expression in fragile X knockout mice
Adusei, D. C., Pacey, L. K. K., Chen, D., & Hampson, D. R. (2010) · Neuropharmacology · doi:10.1016/j.neuropharm.2010.05.002 · Cited by 139 · see citing works
Deng, P., Sojka, D., & Klyachko, V. A. (2011) · Journal of Neuroscience · doi:10.1523/jneurosci.2021-11.2011 · Cited by 139 · see citing works
A mouse model of the human Fragile X syndrome I304N mutation
Zang, J. B., Nosyreva, E. D., Spencer, C. M., Volk, L. J., Musunuru, K., Zhong, R., Stone, E. F., Yuva-Paylor, L. A., Huber, K. M., Paylor, R., et al. (2009) · PLoS Genetics · doi:10.1371/journal.pgen.1000758 · Cited by 138 · see citing works
Self-citationSuvrathan, A., Hoeffer, C. A., Wong, H., Klann, E., & Chattarji, S. (2010) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1002262107 · Cited by 137 · see citing works
Self-citationStrumbos, J. G., Brown, M. R., Kronengold, J., Polley, D. B., & Kaczmarek, L. K. (2010) · The Journal of Neuroscience · doi:10.1523/jneurosci.1125-10.2010 · Cited by 135 · see citing works
Altered Hippocampal Synaptic Plasticity in the <i>Fmr1</i> Gene Family Knockout Mouse Models
Zhang, J., Hou, L., Klann, E., & Nelson, D. L. (2009) · Journal of Neurophysiology · doi:10.1152/jn.90558.2008 · Cited by 126 · see citing works
Self-citationPharmacological treatment of fragile X syndrome with GABAergic drugs in a knockout mouse model
Heulens, I., D’Hulst, C., Dam, D. V., Deyn, P. P. D., & Kooy, R. F. (2012) · Behavioural Brain Research · doi:10.1016/j.bbr.2012.01.031 · Cited by 125 · see citing works
Cytoplasmic RNA-Binding Proteins and the Control of Complex Brain FunctionPreprint
Darnell, J. C., & Richter, J. D. (2012) · Cold Spring Harbor Perspectives in Biology · doi:10.1101/cshperspect.a012344 · Cited by 124 · see citing works
Self-citationSynaptic Plasticity and Translation InitiationPreprint
Klann, E., Antion, M. D., Banko, J. L., & Hou, L. (2004) · Learning & Memory · doi:10.1101/lm.79004 · Cited by 112 · see citing works
Self-citationAltered protein synthesis is a trigger for long-term memory formation
Klann, E., & Sweatt, J. D. (2007) · Neurobiology of Learning and Memory · doi:10.1016/j.nlm.2007.08.009 · Cited by 109 · see citing works
Self-citationGenetic manipulation of STEP reverses behavioral abnormalities in a fragile X syndrome mouse model
Goebel‐Goody, S. M., Wilson‐Wallis, E. D., Royston, S., Tagliatela, S. M., Naegele, J. R., & Lombroso, P. J. (2012) · Genes, Brain and Behavior · doi:10.1111/j.1601-183x.2012.00781.x · Cited by 108 · see citing works
Hanson, J. E., & Madison, D. V. (2007) · Journal of Neuroscience · doi:10.1523/jneurosci.4717-06.2007 · Cited by 103 · see citing works
Altered maturation of the primary somatosensory cortex in a mouse model of fragile X syndrome
Till, S. M., Wijetunge, L. S., Seidel, V. G., Harlow, E., Wright, A. K., Bagni, C., Contractor, A., Gillingwater, T. H., & Kind, P. C. (2012) · Human Molecular Genetics · doi:10.1093/hmg/dds030 · Cited by 91 · see citing works
Siller, S. S., & Broadie, K. S. (2011) · Disease Models & Mechanisms · doi:10.1242/dmm.008045 · Cited by 86 · see citing works
The Cyclic AMP Cascade Is Altered in the Fragile X Nervous System
Kelley, D. J., Davidson, R. J., Elliott, J. L., Lahvis, G. P., Yin, J. C. P., & Bhattacharyya, A. (2007) · PLoS ONE · doi:10.1371/journal.pone.0000931 · Cited by 81 · see citing works
Groß, C., & Bassell, G. J. (2011) · Molecular Medicine · doi:10.2119/molmed.2011.00363 · Cited by 69 · see citing works
Presynaptic translation: stepping out of the postsynaptic shadow
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- Authors
- Darnell, J. C., & Klann, E.
- Affiliations
- Rockefeller University (US)
- New York University (US)
- Corresponding author
- Darnell, Jennifer C
- Klann, Eric
- Venue
- Nature Neuroscience
- Published
- 2013-04-14
- Volume
- 16
- Issue
- 11
- Pages
- 1530-1536
- ISSN
- 1097-6256 (ISSN-L)
- 1546-1726
- PMC
- PMC3999698
- OpenAlex
- W2001215117
- Cited by
- 491
- References
- 98
- Citations by year
- 2026: 21 · 2025: 21 · 2024: 33 · 2023: 26 · 2022: 44 · 2021: 35 · 2020: 37 · 2019: 41 · 2018: 46 · 2017: 41 · 2016: 41 · 2015: 53 · 2014: 37 · 2013: 13
- FWCI
- 5.89
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Nature Neuroscience (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- U.S. Department of Defense
- National Institutes of Health
- National Institute of Neurological Disorders and Stroke (NS-047384, R01NS047384)
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (HD040647, R01HD040647)
- Congressionally Directed Medical Research Programs
- 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 › Neuroscience › Cellular and Molecular Neuroscience › Neuroscience and Neuropharmacology Research
- Keywords
- FMRP, fragile X syndrome, translational regulation, mRNA-binding proteins, ribosome stalling, synaptic plasticity, dendritic spines, cognitive impairment, autism spectrum disorder, therapeutic target, small molecules, RNA-binding proteins
- MeSH terms
- Animals, Fragile X Syndrome, Humans, Intellectual Disability, Neuronal Plasticity, RNA, Messenger, Protein Biosynthesis, Receptors, Metabotropic Glutamate, Fragile X Mental Retardation Protein, Fragile X Messenger Ribonucleoprotein 1
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
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