Pathological plasticity in fragile X syndrome.
Open accessAbstract
Deficits in neuronal plasticity are common hallmarks of many neurodevelopmental disorders. In the case of fragile-X syndrome (FXS), disruption in the function of a single gene,FMR1, results in a variety of neurological consequences directly related to problems with the development, maintenance, and capacity of plastic neuronal networks. In this paper, we discuss current research illustrating the mechanisms underlying plasticity deficits in FXS. These processes include synaptic, cell intrinsic, and homeostatic mechanisms both dependent on and independent of abnormal metabotropic glutamate receptor transmission. We place particular emphasis on how identified deficits may play a role in developmental critical periods to produce neuronal networks with permanently decreased capacity to dynamically respond to changes in activity central to learning, memory, and cognition in patients with FXS. Characterizing early developmental deficits in plasticity is fundamental to develop therapies that not only treat symptoms but also minimize the developmental pathology of the disease.
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
Hallmarks of neural circuit function in FXS include hyperexcitability and plasticity defects.
“Hallmarks of neural circuit function in FXS include hyperexcitability and plasticity defects (Martin and Huntsman, 2012).”
From Astrocytes in fragile X syndrome (2024) · cites this paper as Martin and Huntsman, 2012
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Cites
The period of susceptibility to the physiological effects of unilateral eye closure in kittens
Hubel, D. H., & Wiesel, T. N. (1970) · The Journal of Physiology · doi:10.1113/jphysiol.1970.sp009022 · Cited by 3,030 · see citing works
Fu, Y.-H., Kuhl, D. P. A., Pizzuti, A., Pieretti, M., Sutcliffe, J. S., Richards, S., Verkert, A. J. M. H., Holden, J. J. A., Fenwick, R. G., Warren, S. T., et al. (1991) · Cell · doi:10.1016/0092-8674(91)90283-5 · Cited by 2,080 · 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
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
Gasparini, F., Lingenhöhl, K., Stoehr, N., Flor, P. J., Heinrich, M., Vranesic, I., Biollaz, M., Allgeier, H., Heckendorn, R., Urwyler, S., et al. (1999) · Neuropharmacology · doi:10.1016/s0028-3908(99)00082-9 · Cited by 725 · 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 · cited 10× · see citing works
“ImGluR(V) upregulation requires phospholipase C β1, outlasts acute mGluR activation, is protein synthesis-dependent, specifically tyrosine kinase-ERK signaling pathway-dependent, and blockade of the current suppresses DHPG-induced epileptogenesis [74].”
BackgroundInfluentialHOMEOSTATIC CONTROL OF NEURAL ACTIVITY: From Phenomenology to Molecular Design
Davis, G. W. (2006) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.28.061604.135751 · Cited by 543 · 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
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
Sugiyama, S., Di Nardo, A. A., Aizawa, S., Matsuo, I., Volovitch, M., Prochiantz, A., & Hensch, T. K. (2008) · Cell · doi:10.1016/j.cell.2008.05.054 · Cited by 491 · see citing works
Tissue specific expression of FMR-1 provides evidence for a functional role in fragile X syndrome
Hinds, H. L., Ashley, C. T., Sutcliffe, J. S., Nelson, D. L., Warren, S. T., Housman, D. E., & Schalling, M. (1993) · Nature Genetics · doi:10.1038/ng0193-36 · Cited by 396 · 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
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
Paradee, W., Melikian, H. E., Rasmussen, D. L., Kenneson, A., Conn, P. J., & Warren, S. T. (1999) · Neuroscience · doi:10.1016/s0306-4522(99)00285-7 · Cited by 305 · see citing works
Weiler, I. J., Spangler, C. C., Klintsova, A. Y., Grossman, A. W., Kim, S. H., Bertaina-Anglade, V., Khaliq, H., de Vries, F. E., Lambers, F. A. E., Hatia, F., et al. (2004) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0407533101 · Cited by 210 · see citing works
Circuit and Plasticity Defects in the Developing Somatosensory Cortex of<i>Fmr1</i>Knock-Out Mice
Bureau, I., Shepherd, G. M. G., & Svoboda, K. (2008) · The Journal of Neuroscience · doi:10.1523/jneurosci.1076-08.2008 · Cited by 201 · see citing works
Maghsoodi, B., Poon, M. M., Nam, C. I., Aoto, J., Ting, P., & Chen, L. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0804801105 · Cited by 138 · see citing works
Musumeci, S. A., Calabrese, G., Bonaccorso, C. M., D'Antoni, S., Brouwer, J. R., Bakker, C. E., Elia, M., Ferri, R., Nelson, D. L., Oostra, B. A., et al. (2007) · Experimental Neurology · doi:10.1016/j.expneurol.2006.08.007 · Cited by 70 · see citing works
Flagged first, then by citations
Research works printed but not linked: 112
- [1]de Vries BBA Halley DJJ Oostra BA Niermeijer MF The fragile X syndrome Journal of Medical Genetics 1998 35 7 579 589 2-s2.0-0031812608 9678703 PMC1051369
- [2]Hagerman RJ Berry-Kravis E Kaufmann WE Advances in the treatment of fragile x Syndrome Pediatrics 2009 123 1 378 390 2-s2.0-59449085928 19117905 PMC2888470
- [3]Tranfaglia MR The psychiatric presentation of fragile x: evolution of the diagnosis and treatment of the psychiatric comorbidities of fragile X syndrome Developmental Neuroscience 2011 33 5 337 348 21893938
- [4]Verkerk AJMH Pieretti M Sutcliffe JS Identification of a gene (FMR-1) containing a CGG repeat coincident with a breakpoint cluster region exhibiting length variation in fragile X syndrome Cell 1991 65 5 905 914 2-s2.0-0025905795 1710175
- [6]Wang H Ku L Osterhout DJ Developmentally-programmed FMRP expression in oligodendrocytes: a potential role of FMRP in regulating translation in oligodendroglia progenitors Human Molecular Genetics 2004 13 1 79 89 2-s2.0-0346656871 14613971
- [7]Olmos-Serrano JL Paluszkiewicz SM Martin BS Kaufmann WE Corbin JG Huntsman MM Defective GABAergic neurotransmission and pharmacological rescue of neuronal hyperexcitability in the amygdala in a mouse model of fragile X syndrome Journal of Neuroscience 2010 30 29 9929 9938 2-s2.0-77954851827 20660275 PMC2948869
- [8]Devys D Lutz Y Rouyer N Bellocq JP Mandel JL The FMR-1 protein is cytoplasmic, most abundant in neurons and appears normal in carriers of a fragile X premutation Nature Genetics 1993 4 4 335 340 2-s2.0-0027176361 8401578
- [9]Feng Y Absher D Eberhart DE Brown V Malter HE Warren ST FMRP associates with polyribosomes as an mRNP, and the I304N mutation of severe fragile X syndrome abolishes this association Molecular Cell 1997 1 1 109 118 2-s2.0-0031310667 9659908
- [11]Antar LN Li C Zhang H Carroll RC Bassell GJ Local functions for FMRP in axon growth cone motility and activity-dependent regulation of filopodia and spine synapses Molecular and Cellular Neuroscience 2006 32 1-2 37 48 2-s2.0-33744966575 16631377
- [12]Li Z Zhang Y Ku L Wilkinson KD Warren ST Feng Y The fragile X mental retardation protein inhibits translation via interacting with mRNA Nucleic Acids Research 2001 29 11 2276 2283 2-s2.0-0035368955 11376146 PMC55699
- [13]Christie SB Akins MR Schwob JE Fallon JR The FXG: a presynaptic Fragile X granule expressed in a subset of developing brain circuits Journal of Neuroscience 2009 29 5 1514 1524 2-s2.0-59649126241 19193898 PMC2746438
- [14]Laggerbauer B Ostareck D Keidel EM Ostareck-Lederer A Fischer U Evidence that fragile X mental retardation protein is a negative regulator of translation Human Molecular Genetics 2001 10 4 329 338 2-s2.0-0035864826 11157796
- [15]Huber KM Gallagher SM Warren ST Bear MF Altered synaptic plasticity in a mouse model of fragile X mental retardation Proceedings of the National Academy of Sciences of the United States of America 2002 99 11 7746 7750 2-s2.0-0037188502 12032354 PMC124340
- [16]Brown V Jin P Ceman S Microarray identification of FMRP-associated brain mRNAs and altered mRNA translational profiles in fragile X syndrome Cell 2001 107 4 477 487 2-s2.0-18044379515 11719188
- [17]Darnell JC Mostovetsky O Darnell RB FMRP RNA targets: identification and validation Genes, Brain and Behavior 2005 4 6 341 349 2-s2.0-17444417737 16098133
- [19]Bassell GJ Warren ST Fragile X syndrome: loss of local mRNA regulation alters synaptic development and function Neuron 2008 60 2 201 214 2-s2.0-53849110899 18957214 PMC3691995
- [20]Berry-Kravis E Epilepsy in fragile X syndrome Developmental Medicine and Child Neurology 2002 44 11 724 728 2-s2.0-0036827976 12418611
- [21]Hagerman PJ Stafstrom CE Origins of epilepsy in fragile X syndrome Epilepsy Currents 2009 9 4 108 112 19693328 PMC2728488
- [22]Miller LJ McIntosh DN McGrath J Electrodermal responses to sensory stimuli in individuals with fragile X syndrome: a preliminary report American Journal of Medical Genetics 1999 83 4 268 279 10208160
- [23]Musumeci SA Hagerman RJ Ferri R Epilepsy and EEG findings in males with fragile X syndrome Epilepsia 1999 40 8 1092 1099 2-s2.0-0032773639 10448821
- [24]Chonchaiya W Schneider A Hagerman RJ Fragile X: a family of disorders Advances in Pediatrics 2009 56 1 165 186 2-s2.0-72149108442 19968948 PMC2921504
- [25]Bakker CE Verheij C Willemsen R Fmr1 knockout mice: a model to study fragile X mental retardation Cell 1994 78 1 23 33 2-s2.0-0028246435 8033209
- [26]Dölen G Osterweil E Rao BSS Correction of fragile X syndrome in mice Neuron 2007 56 6 955 962 2-s2.0-37049032616 18093519 PMC2199268
- [30]Irwin SA Galvez R Greenough WT Dendritic spine structural anomalies in fragile-X mental retardation syndrome Cerebral Cortex 2000 10 10 1038 1044 2-s2.0-0033797832 11007554
- [32]Pan L Broadie KS Drosophila fragile X mental retardation protein and metabotropic glutamate receptor a convergently regulate the synaptic ratio of ionotropic glutamate receptor subclasses Journal of Neuroscience 2007 27 45 12378 12389 2-s2.0-36048947587 17989302 PMC6673270
- [33]Pan L Woodruff E Liang P Broadie K Mechanistic relationships between Drosophila fragile X mental retardation protein and metabotropic glutamate receptor A signaling Molecular and Cellular Neuroscience 2008 37 4 747 760 2-s2.0-41149114485 18280750 PMC3989938
- [34]Repicky S Broadie K Metabotropic glutamate receptor-mediated use-dependent down-regulation of synaptic excitability involves the fragile X mental retardation protein Journal of Neurophysiology 2009 101 2 672 687 2-s2.0-61349164873 19036865 PMC2657068
- [37]de Vrij FMS Levenga J van der Linde HC Rescue of behavioral phenotype and neuronal protrusion morphology in Fmr1 KO mice Neurobiology of Disease 2008 31 1 127 132 2-s2.0-44949125523 18571098 PMC2481236
- [38]Aschrafi A Cunningham BA Edelman GM Vanderklish PW The fragile X mental retardation protein and group I metabotropic glutamate receptors regulate levels of mRNA granules in brain Proceedings of the National Academy of Sciences of the United States of America 2005 102 6 2180 2185 2-s2.0-13844316424 15684045 PMC548595
- [39]McBride SMJ Choi CH Wang Y Pharmacological rescue of synaptic plasticity, courtship behavior, and mushroom body defects in a Drosophila model of Fragile X syndrome Neuron 2005 45 5 753 764 2-s2.0-20044388322 15748850
- [40]Michalon A Sidorov M Ballard TM Chronic pharmacological mGlu5 inhibition corrects fragile X in adult mice Neuron 2012 74 1 49 56 22500629 PMC8822597
- [42]Huber KM Kayser MS Bear MF Role for rapid dendritic protein synthesis in hippocampal mGluR- dependent long-term depression Science 2000 288 5469 1254 1256 2-s2.0-0034685813 10818003
- [44]Koekkoek SKE Yamaguchi K Milojkovic BA Deletion of FMR1 in purkinje cells enhances parallel fiber LTD, enlarges spines, and attenuates cerebellar eyelid conditioning in fragile X syndrome Neuron 2005 47 3 339 352 2-s2.0-23044503253 16055059
- [45]Godfraind JM Reyniers E De Boulle K Long-term potentiation in the hippocampus of fragile X knockout mice American Journal of Medical Genetics 1996 64 2 246 251 8844057
- [47]Li J Pelletier MR Perez Velazquez JL Carlen PL Reduced cortical synaptic plasticity and GluR1 expression associated with fragile X mental retardation protein deficiency Molecular and Cellular Neuroscience 2002 19 2 138 151 2-s2.0-0036198673 11860268
- [48]Wilson BM Cox CL Absence of metabotropic glutamate receptor-mediated plasticity in the neocortex of fragile X mice Proceedings of the National Academy of Sciences of the United States of America 2007 104 7 2454 2459 2-s2.0-33947119365 17287348 PMC1892931
- [49]Suvrathan A Hoeffer CA Wong H Klann E Chattarji S Characterization and reversal of synaptic defects in the amygdala in a mouse model of fragile X syndrome Proceedings of the National Academy of Sciences of the United States of America 2010 107 25 11591 11596 2-s2.0-77954858360 20534533 PMC2895119
- [50]Zhao M-G Toyoda H Ko SW Ding HK Wu LJ Zhuo M Deficits in trace fear memory and long-term potentiation in a mouse model for fragile X syndrome Journal of Neuroscience 2005 25 32 7385 7392 2-s2.0-23744492534 16093389 PMC6725289
- [51]Lee HY Ge WP Huang W Bidirectional regulation of dendritic voltage-gated potassium channels by the fragile X mental retardation protein Neuron 2011 72 4 630 642 22099464 PMC3433402
- [52]Till SM Li HL Miniaci MC Kandel ER Choi YB A presynaptic role for FMRP during protein synthesis-dependent long-term plasticity in Aplysia Learning and Memory 2011 18 1 39 48 2-s2.0-78650987058 21177378 PMC3023967
- [53]Deng PY Sojka D Klyachko VA Abnormal presynaptic short-term plasticity and information processing in a mouse model of fragile X syndrome Journal of Neuroscience 2011 31 30 10971 10982 2-s2.0-79960955668 21795546 PMC6623101
- [54]Bacci A Huguenard JR Prince DA Long-lasting self-inhibition of neocortical interneurons mediated by endocannabinoids Nature 2004 431 7006 312 316 2-s2.0-4644350311 15372034
- [55]Wilson RI Nicoll RA Neuroscience: endocannabinoid signaling in the brain Science 2002 296 5568 678 682 2-s2.0-0037177601 11976437
- [56]Misgeld U Bijak M Jarolimek W A physiological role for GABA(B) receptors and the effects of baclofen in the mammalian central nervous system Progress in Neurobiology 1995 46 4 423 462 2-s2.0-0028983775 8532848
- [57]Varma N Carlson GC Ledent C Alger BE Metabotropic glutamate receptors drive the endocannabinoid system in hippocampus The Journal of Neuroscience 2001 21 24 p. RC188 2-s2.0-0035893233 11734603 PMC6763031
- [58]Gibson JR Bartley AF Hays SA Huber KM Imbalance of neocortical excitation and inhibition and altered UP states reflect network hyperexcitability in the mouse model of fragile X syndrome Journal of Neurophysiology 2008 100 5 2615 2626 2-s2.0-53849100522 18784272 PMC2585391
- [59]Zhang L Alger BE Enhanced endocannabinoid signaling elevates neuronal excitability in fragile X syndrome Journal of Neuroscience 2010 30 16 5724 5729 2-s2.0-77951528668 20410124 PMC2906112
- [60]Lee S-H Földy C Soltesz I Distinct endocannabinoid control of GABA release at perisomatic and dendritic synapses in the hippocampus Journal of Neuroscience 2010 30 23 7993 8000 2-s2.0-77953505047 20534847 PMC2904437
- [61]Trettel J Levine ES Cannabinoids depress inhibitory synaptic inputs received by layer 2/3 pyramidal neurons of the neocortex Journal of Neurophysiology 2002 88 1 534 539 2-s2.0-0036314517 12091577
- [62]Bacci A Huguenard JR Prince DA Modulation of neocortical interneurons: extrinsic influences and exercises in self-control Trends in Neurosciences 2005 28 11 602 610 2-s2.0-26944443841 16139371
- [63]Fanselow EE Richardson KA Connors BW Selective, state-dependent activation of somatostatin-expressing inhibitory interneurons in mouse neocortex Journal of Neurophysiology 2008 100 5 2640 2652 2-s2.0-57049178384 18799598 PMC2585405
- [64]Beierlein M Gibson JR Connors BW A network of electrically coupled interneurons drives synchronized inhibition in neocortex Nature Neuroscience 2000 3 9 904 910 2-s2.0-0033860986 10966621
- [65]Paluszkiewicz SM Olmos-Serrano JL Corbin JG Huntsman MM Impaired inhibitory control of cortical synchronization in fragile X syndrome J Neurophysiol 2011 106 5 2264 2272 21795626 PMC3214096
- [66]Fanselow EE Connors BW The roles of somatostatin-expressing (GIN) and fast-spiking inhibitory interneurons in up-down states of mouse neocortex Journal of Neurophysiology 2010 104 2 596 606 2-s2.0-77955955386 20538767 PMC2934925
- [67]Long MA Cruikshank SJ Jutras MJ Connors BW Abrupt maturation of a spike-synchronizing mechanism in neocortex Journal of Neuroscience 2005 25 32 7309 7316 2-s2.0-23744505382 16093380 PMC6725294
- [68]Merlin LR Wong RKS Role of group I metabotropic glutamate receptors in the patterning of epileptiform activities in vitro Journal of Neurophysiology 1997 78 1 539 544 2-s2.0-0030809272 9242303
- [69]Merlin LR Bergold PJ Wong RKS Requirement of protein synthesis for group I mGluR-mediated induction of epileptiform discharges Journal of Neurophysiology 1998 80 2 989 993 2-s2.0-0031813129 9705485
- [70]Lieb JP Engel J Brown WJ Neuropathological findings following temporal lobectomy related to surface and deep EEG patterns Epilepsia 1981 22 5 539 549 2-s2.0-0019417772 7285882
- [71]Taylor GW Merlin LR Wong RKS Synchronized oscillations in hippocampal CA3 neurons induced by metabotropic glutamate receptor activation Journal of Neuroscience 1995 15 12 8039 8052 2-s2.0-0029588377 8613741 PMC6577924
- [72]Zhao W Bianchi R Wang M Wong RKS Extracellular signal-regulated kinase 1/2 is required for the induction of group I metabotropic glutamate receptor-mediated epileptiform discharges Journal of Neuroscience 2004 24 1 76 84 2-s2.0-0842344418 14715940 PMC6729577
- [73]Lee AC Wong RKS Chuang SC Shin HS Bianchi R Role of synaptic metabotropic glutamate receptors in epileptiform discharges in hippocampal slices Journal of Neurophysiology 2002 88 4 1625 1633 2-s2.0-0036796648 12364493
- [74]Bianchi R Chuang SC Zhao W Young SR Wong RKS Cellular plasticity for group I mGluR-mediated epileptogenesis Journal of Neuroscience 2009 29 11 3497 3507 2-s2.0-63849232993 19295155 PMC2692254
- [75]Wong RKS Chuang SC Bianchi R Plasticity mechanisms underlying mGluR-induced epileptogenesis Advances in Experimental Medicine and Biology 2004 548 69 75 2-s2.0-3242778548 15250586
- [76]Chuang SC Bianchi R Wong RKS Group I mGluR activation turns on a voltage-gated inward current in hippocampal pyramidal cells Journal of Neurophysiology 2000 83 5 2844 2853 2-s2.0-0034078492 10805682
- [77]Chuang SC Bianchi R Kim D Shin HS Wong RKS Group I metabotropic glutamate receptors elicit epileptiform discharges in the hippocampus through PLCβ1 signaling Journal of Neuroscience 2001 21 16 6387 6394 2-s2.0-0035882285 11487662 PMC6763182
- [78]Shang Y Wang H Mercaldo V Li X Chen T Zhuo M Fragile X mental retardation protein is required for chemically-induced long-term potentiation of the hippocampus in adult mice Journal of Neurochemistry 2009 111 3 635 646 2-s2.0-70349909566 19659572
- [79]Chuang S-C Zhao W Bauchwitz R Yan Q Bianchi R Wong RKS Prolonged epileptiform discharges induced by altered group I metabotropic glutamate receptor-mediated synaptic responses in hippocampal slices of a fragile X mouse model Journal of Neuroscience 2005 25 35 8048 8055 2-s2.0-24344445896 16135762 PMC6725444
- [80]Zhong J Chuang SC Bianchi R BC1 regulation of metabotropic glutamate receptor-mediated neuronal excitability Journal of Neuroscience 2009 29 32 9977 9986 2-s2.0-68849091812 19675232 PMC2866649
- [81]Zhong J Chuang SC Bianchi R Regulatory BC1 RNA and the fragile X mental retardation protein: convergent functionality in brain PLoS ONE 2010 5 11 2-s2.0-78649524251 e15509 PMC2990754 21124905
- [82]Dahlhaus R El-Husseini A Altered neuroligin expression is involved in social deficits in a mouse model of the fragile X syndrome Behavioural brain research 2010 208 1 96 105 2-s2.0-77951897264 19932134
- [83]Curia G Papouin T Séguéla P Avoli M Downregulation of tonic GABAergic inhibition in a mouse model of fragile X syndrome Cerebral Cortex 2009 19 7 1515 1520 2-s2.0-67649427508 18787232 PMC4873279
- [84]Turrigiano GG Nelson SB Homeostatic plasticity in the developing nervous system Nature Reviews Neuroscience 2004 5 2 97 107 2-s2.0-0742323527 14735113
- [87]Aoto J Nam CI Poon MM Ting P Chen L Synaptic signaling by all-trans retinoic acid in homeostatic synaptic plasticity Neuron 2008 60 2 308 320 2-s2.0-53849098397 18957222 PMC2634746
- [88]Soden ME Chen L Fragile X protein FMRP is required for homeostatic plasticity and regulation of synaptic strength by retinoic acid Journal of Neuroscience 2010 30 50 16910 16921 2-s2.0-78650321794 21159962 PMC3073636
- [89]LeBlanc JJ Fagiolini M Autism: a “critical period” disorder? Neural Plasticity 2011 2011 17 pages 921680 PMC3150222 21826280
- [90]Hensch TK Critical period regulation Annual Review of Neuroscience 2004 27 549 579 2-s2.0-3943087045 15217343
- [92]Harrison RV Gordon KA Mount RJ Is there a critical period for cochlear implantation in congenitally deaf children? Analyses of hearing and speech perception performance after implantation Developmental Psychobiology 2005 46 3 252 261 2-s2.0-17844383723 15772969
- [93]Popescu MV Polley DB Monaural deprivation disrupts development of binaural selectivity in auditory midbrain and cortex Neuron 2010 65 5 718 731 2-s2.0-77649148710 20223206 PMC2849994
- [94]Fox K A critical period for experience-dependent synaptic plasticity in rat barrel cortex Journal of Neuroscience 1992 12 5 1826 1838 2-s2.0-0026550383 1578273 PMC6575898
- [95]Van Der Loos H Woolsey TA Somatosensory cortex: structural alterations following early injury to sense organs Science 1973 179 4071 395 398 2-s2.0-0015936160 4682966
- [96]Banks MS Aslin RN Letson RD Sensitive period for the development of human binocular vision Science 1975 190 4215 675 677 2-s2.0-0016766752 1188363
- [97]Berardi N Pizzorusso T Maffei L Critical periods during sensory development Current Opinion in Neurobiology 2000 10 1 138 145 2-s2.0-0033969699 10679428
- [98]Baranek GT Roberts JE David FJ Developmental trajectories and correlates of sensory processing in young boys with fragile X syndrome Physical and Occupational Therapy in Pediatrics 2008 28 1 79 98 2-s2.0-42449089448 18399048
- [99]Rogers SJ Hepburn S Wehner E Parent reports of sensory symptoms in toddlers with autism and those with other developmental disorders Journal of Autism and Developmental Disorders 2003 33 6 631 642 2-s2.0-0348048581 14714932
- [100]Abitbol M Menini C Delezoide AL Rhyner T Vekemans M Mallet J Nucleus basalis magnocellularis and hippocampus are the major sites of FMR-1 expression in the human fetal brain Nature Genetics 1993 4 2 147 153 2-s2.0-0027300283 8348153
- [101]Agulhon C Blanchet P Kobetz A Expression of FMR1, FXR1, and FXR2 genes in human prenatal tissues Journal of Neuropathology & Experimental Neurology 1999 58 8 867 880 10446811
- [102]Tamanini F Willemsen R Van Unen L Differential expression of FMR1, FXR1 and FXR2 proteins in human brain and testis Human Molecular Genetics 1997 6 8 1315 1322 2-s2.0-0030753950 9259278
- [103]Harlow EG Till SM Russell TA Wijetunge LS Kind P Contractor A Critical period plasticity is disrupted in the barrel cortex of Fmr1 knockout mice Neuron 2010 65 3 385 398 2-s2.0-75949119188 20159451 PMC2825250
- [104]Todd PK Malter JS Mack KJ Whisker stimulation-dependent translation of FMRP in the barrel cortex requires activation of type I metabotropic glutamate receptors Molecular Brain Research 2003 110 2 267 278 2-s2.0-0037456073 12591163
- [105]Boer K Encha-Razavi F Sinico M Aronica E Differential distribution of group I metabotropic glutamate receptors in developing human cortex Brain Research C 2010 1324 24 33 2-s2.0-77949917437 20149785
- [106]Catania MV Landwehrmeyer GB Testa CM Standaert DG Penney JB Young AB Metabotropic glutamate receptors are differentially regulated during development Neuroscience 1994 61 3 481 495 7969925
- [107]Castiglione M Calafiore M Costa L Sortino MA Nicoletti F Copani A Group I metabotropic glutamate receptors control proliferation, survival and differentiation of cultured neural progenitor cells isolated from the subventricular zone of adult mice Neuropharmacology 2008 55 4 560 567 2-s2.0-49949100934 18603270
- [108]Di Giorgi-Gerevini V Melchiorri D Battaglia G Endogenous activation of metabotropic glutamate receptors supports the proliferation and survival of neural progenitor cells Cell Death and Differentiation 2005 12 8 1124 1133 2-s2.0-23044514252 15947794
- [109]López-Bendito G Shigemoto R Fairén A Luján R Differential distribution of group I metabotropic glutamate receptors during rat cortical development Cerebral Cortex 2002 12 6 625 638 2-s2.0-0036091128 12003862
- [110]Paluszkiewicz SM Martin BS Huntsman MM Fragile X syndrome: the GABAergic system and circuit dysfunction Developmental Neuroscience 2011 33 5 349 364 21934270 PMC3254035
- [111]Adusei DC Pacey LKK Chen D Hampson DR Early developmental alterations in GABAergic protein expression in fragile X knockout mice Neuropharmacology 2010 59 3 167 171 2-s2.0-77954143021 20470805
- [113]D’Hulst C Heulens I Brouwer JR Expression of the GABAergic system in animal models for fragile X syndrome and fragile X associated tremor/ataxia syndrome (FXTAS) Brain Research C 2009 1253 176 183 2-s2.0-58249135425 19070606
- [114]Gantois I Vandesompele J Speleman F Expression profiling suggests underexpression of the GABA(A) receptor subunit delta in the fragile X knockout mouse model Neurobiology of Disease 2006 21 2 346 357 16199166
- [115]Fagiolini M Katagiri H Miyamoto H Separable features of visual cortical plasticity revealed by N-methyl-D-aspartate receptor 2A signaling Proceedings of the National Academy of Sciences of the United States of America 2003 100 5 2854 2859 2-s2.0-0037418328 12591944 PMC151430
- [116]Fagiolini M Fritschy JM Löw K Möhler H Rudolph U Hensch TK Specific GABAA circuits for visual cortical plasticity Science 2004 303 5664 1681 1683 2-s2.0-1542408736 15017002
- [117]Hensch TK Fagiolini M Mataga N Stryker MP Baekkeskov S Kash SF Local GABA circuit control of experience-dependent plasticity in developing visual cortex Science 1998 282 5393 1504 1508 2-s2.0-0032553477 9822384 PMC2851625
- [118]Frenkel MY Bear MF How monocular deprivation shifts ocular dominance in visual cortex of young mice Neuron 2004 44 6 917 923 2-s2.0-10444251754 15603735
- [119]Woolsey TA Van der Loos H The structural organization of layer IV in the somatosensory region (S I) of mouse cerebral cortex. The description of a cortical field composed of discrete cytoarchitectonic units Brain Research 1970 17 2 205 242 2-s2.0-0014955657 4904874
- [120]Till SM Wijetunge LS Seidel VG Altered maturation of the primary somatosensory cortex in a mouse model of fragile X syndrome Human Molecular Genetics 2012 21 10 2143 2156 22328088
- [122]Dictenberg JB Swanger SA Antar LN Singer RH Bassell GJ A direct role for FMRP in activity-dependent dendritic mRNA transport links filopodial-spine morphogenesis to fragile X syndrome Developmental Cell 2008 14 6 926 939 2-s2.0-44449121279 18539120 PMC2453222
- [124]Chen L Toth M Fragile X mice develop sensory hyperreactivity to auditory stimuli Neuroscience 2001 103 4 1043 1050 2-s2.0-0035804765 11301211
- [125]Krueger DD Bear MF Toward fulfilling the promise of molecular medicine in fragile X syndrome Annual Review of Medicine 2011 62 411 429 2-s2.0-79551594543 PMC3100156 21090964
- [126]Zhu PJ Lovinger DM Developmental alteration of endocannabinoid retrograde signaling in the hippocampus Journal of Neurophysiology 2010 103 2 1123 1129 2-s2.0-76649129302 20007500 PMC2822685
- [127]Turrigiano GG Nelson SB Hebb and homeostasis in neuronal plasticity Current Opinion in Neurobiology 2000 10 3 358 364 2-s2.0-0034131101 10851171
- [128]Huang ZJ Kirkwood A Pizzorusso T BDNF regulates the maturation of inhibition and the critical period of plasticity in mouse visual cortex Cell 1999 98 6 739 755 2-s2.0-0033578688 10499792
- [130]Hays SA Huber KM Gibson JR Altered neocortical rhythmic activity states in Fmr1 KO mice are due to enhanced mGluR5 signaling and involve changes in excitatory circuitry Journal of Neuroscience 2011 31 40 14223 14234 21976507 PMC3207280
- [131]Vislay-Meltzer RL Kratovac S Olmos-Serrano L The development and consequences of inhibitory deficits in the fragile X amygdala Proceedings of the 41st Annual Society For Neuroscience Meeting 2011 Washington, DC, USA Poster#774.02/O12
Cited by
Sorted by citations
Mammalian/mechanistic target of rapamycin (mTOR) complexes in neurodegeneration
Querfurth, H., & Lee, H.-K. (2021) · Molecular Neurodegeneration · doi:10.1186/s13024-021-00428-5 · Cited by 288 · see citing works
Merino-Serrais, P., Benavides-Piccione, R., Blazquez-Llorca, L., Kastanauskaite, A., Rábano, A., Avila, J., & DeFelipe, J. (2013) · Brain · doi:10.1093/brain/awt088 · Cited by 134 · see citing works
Siegel, J. J., Chitwood, R. A., Ding, J. M., Payne, C., Taylor, W., Gray, R., Zemelman, B. V., & Johnston, D. (2017) · The Journal of Neuroscience · doi:10.1523/jneurosci.0571-17.2017 · Cited by 27 · see citing works
Astrocytes in fragile X syndrome
Talvio, K., & Castrén, M. L. (2024) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2023.1322541 · Cited by 8 · see citing works
“Hallmarks of neural circuit function in FXS include hyperexcitability and plasticity defects (Martin and Huntsman, 2012).”
Background
- Authors
- Martin, B. S., & Huntsman, M. M.
- Affiliations
- Children's National (US)
- Corresponding author
- Huntsman, Molly M.
- ORCID
- Huntsman, Molly M.
- Venue
- Neural Plasticity
- Publisher
- Wiley
- Published
- 2012
- Volume
- 2012
- Pages
- 1-12
- ISSN
- 1687-5443 (ISSN-L)
- 2090-5904
- PMC
- PMC3395158
- OpenAlex
- W2096225153
- Cited by
- 57
- References
- 19
- Citations by year
- 2026: 6 · 2025: 1 · 2024: 2 · 2023: 5 · 2022: 3 · 2021: 8 · 2020: 8 · 2019: 2 · 2018: 1 · 2017: 2 · 2016: 3 · 2015: 3 · 2014: 6 · 2013: 7
- FWCI
- 0.63
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- Neural Plasticity (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Funders
- National Institute of Neurological Disorders and Stroke (R01 NS053719)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Neuroscience and Neuropharmacology Research
- Life Sciences › Neuroscience › Cognitive Neuroscience › Autism Spectrum Disorder Research
- Keywords
- fragile X syndrome, FMR1 gene, neuroplasticity, synaptic plasticity, metabotropic glutamate receptors, homeostatic plasticity, critical developmental periods, neurodevelopmental disorders, learning and memory impairment, cognitive impairment
- MeSH terms
- Fragile X Syndrome, Homeostasis, Humans, Nerve Net, Neuronal Plasticity, Receptors, Glutamate, Receptors, Cannabinoid, Adaptor Proteins, Signal Transducing, 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.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review