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Fragile X mouse: strain effects of knockout phenotype and evidence suggesting deficient amygdala function
Abstract
Fragile X syndrome is an X-linked form of mental retardation resulting from the absence of expression of the fragile X mental retardation 1 gene. The encoded protein is a ribosome-associated, RNA-binding protein thought to play a role in translational regulation of selective messenger RNA transcripts. A knockout mouse has been described that exhibits subtle deficits in spatial learning but normal early-phase long-term potentiation. We expanded these studies by examination of late-phase hippocampal long-term potentiation, the protein synthesis-dependent form of long-term potentiation, in the Fmrl knockout mice. Here, late-phase long-term potentiation was normal, suggesting either that absence of fragile X mental retardation protein has no influence on long-term potentiation or that any influence is too subtle to be detected by this technique. Alternatively, the hippocampus may not be the primary site affected by the absence of this protein. Accordingly, we examined spatial learning in the knockout mice using the hippocampus-dependent Morris water maze. Contrary to earlier reports, near-normal performance was observed. Since the knockout line used in this study has been back-crossed to C57BL/6 for more than 15 generations, whereas the line used in the earlier studies contained a substantial strain 129 contribution, we examined F1 siblings of knockout and 129 crosses. Here, significant but subtle increased swim latencies in reversal trials were observed, in agreement with the previous studies. These data suggest strain differences between C57BL/6 and 129 that influence the Fmrl knockout phenotype. In order to investigate a paradigm less dependent on hippocampal function, the knockout mice were examined using the conditional fear paradigm. Here, the knockout animals displayed significantly less freezing behavior than their wild-type littermates following both contextual and conditional fear stimuli. These data suggest that amygdala disturbances may also be involved in fragile X syndrome.
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“By most accounts Fmr1 KO mice appear to have relatively normal behavior, but research has shown that the behavioral and cognitive deficits of the KO are actually quite subtle and parallel FXS patients (Berry-Kravis et al., 2002; D’Hooge et al., 1997; Paradee et al., 1999; Peier et al., 2000).”
From Oxytocin and vasopressin systems in genetic syndromes and neurodevelopmental disorders (2014) · cites this paper as Paradee et al., 1999
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Items 201–300 of 305
Rett Syndrome and Fragile X Syndrome: Different Etiology With Common Molecular Dysfunctions
Bach, S., Shovlin, S., Moriarty, M. J., Bardoni, B., & Tropea, D. (2021) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2021.764761 · Cited by 25
“…No difference in LTP Godfraind et al., 1996 Fmr1−/Y Hippocampal slices (P35–P56) Paired-pulse facilitation, theta-burst stimulation No difference in LTP Paradee et al., 1999 Fmr1−/Y Somatosensory cortex (P5–P11) LTP stimulation, bumetanide treatment ↑ LTP which is corrected by bumetanide He et…”
BackgroundCoremans, V., Ahmed, T., Balschun, D., D’Hooge, R., Vriese, A. D., Cremer, J., Antonucci, F., Moons, M., Baekelandt, V., Reumers, V., et al. (2010) · BMC Neuroscience · doi:10.1186/1471-2202-11-2 · Cited by 22
“Contextual and auditory-cued fear conditioning [ 67 ,68] was tested in a Plexiglas chamber with a grid floor through which a foot shock could be administered.”
MethodSTDP and mental retardation: dysregulation of dendritic excitability in Fragile X syndrome
Meredith, R. M. (2010) · Frontiers in Synaptic Neuroscience · doi:10.3389/fnsyn.2010.00010 · Cited by 21
“These findings echo data using traditional synaptic plasticity paradigms in Fmr1-KO mice: no significant differences in field LTP were reported initially in the Fmr1-KO mouse model following tetanic stimulation in hippocampus (Godfraind et al., 1996; Paradee et al., 1999; Li et al., 2002).”
Tetraspanin 6: A novel regulator of hippocampal synaptic transmission and long term plasticity
Salas, I. H., Callaerts‐Vegh, Z., Arranz, A. M., Guix, F. X., D’Hooge, R., Esteban, J. A., Strooper, B. D., & Dotti, C. G. (2017) · PLoS ONE · doi:10.1371/journal.pone.0171968 · Cited by 23
New insights into Fragile X syndrome
Pozdnyakova, I., & Regan, L. (2005) · FEBS Journal · doi:10.1111/j.1742-4658.2004.04527.x · Cited by 22
Taurine regulation of short term synaptic plasticity in fragile X mice
Idrissi, A. E., Neuwirth, L. S., & L’Amoreaux, W. J. (2010) · Journal of Biomedical Science · doi:10.1186/1423-0127-17-s1-s15 · Cited by 22
“No changes in paired pulse facilitation in hippocampal circuits have been noted when the interpulse interval was 40 ms [28] or 50 to 1600 ms [27].”
BackgroundGenetic background mutations drive neural circuit hyperconnectivity in a fragile X syndrome model
Kennedy, T., Rinker, D. C., & Broadie, K. S. (2020) · BMC Biology · doi:10.1186/s12915-020-00817-0 · Cited by 19
Is fragile X mental retardation protein involved in activity-induced plasticity of dendritic spines?
Segal, M., Kreher, U., Greenberger, V., & Braun, K. A. (2003) · Brain Research · doi:10.1016/s0006-8993(03)02410-7 · Cited by 21
Comprehensive motor testing in Fmr1-KO mice exposes temporal defects in oromotor coordination.
Roy, S., Zhao, Y., Allensworth, M. L., Farook, M. F., LeDoux, M. S., Reiter, L. T., & Heck, D. H. (2011) · Behavioral Neuroscience · doi:10.1037/a0025920 · Cited by 22
Neuronal RNA‐binding protein HuD regulates addiction‐related gene expression and behavior
Oliver, R. J., Brigman, J. L., Bolognani, F., Allan, A. M., Neisewander, J. L., & Perrone‐Bizzozero, N. I. (2017) · Genes Brain & Behavior · doi:10.1111/gbb.12454 · Cited by 21
“tional response, where both overexpression and knockout of a regulatory gene impairs spatial learning, thus, suggesting that a specific expression pattern of downstream plasticity-associated genes is required for proper learning and memory.(78,79) Adding further”
BackgroundReversal learning paradigm reveals deficits in cognitive flexibility in the Fmr1 knockout male mouse
Nolan, S. O., & Lugo, J. N. (2018) · F1000Research · doi:10.12688/f1000research.14969.1 · Cited by 20
“In any case, the authors should likely revisit this statement, and instead may wish to report Paradee et al. (1999)3 which used fully backcrossed C57BL/6 KO mice.”
BackgroundMethodInfluentialIncreasing our understanding of human cognition through the study of fragile X syndrome
Cook, D., Nuro, E., & Murai, K. K. (2013) · Developmental Neurobiology · doi:10.1002/dneu.22096 · Cited by 22
“Changes in E-LTP at the CA3-CA1 synapse in Fmr1 knockout mice have been observed, but appear to be inconsistent across the literature, perhaps due to differences in FMRP requirement over the course of development (Godfraind et al., 1996; Paradee et al., 1999; Pilpel et al., 2009; Lee et al., 2011).”
BackgroundGenetic modifiers in mice: the example of the fragile X mouse model
Errijgers, V., & Kooy, R. F. (2004) · Cytogenetic and Genome Research · doi:10.1159/000078218 · Cited by 19
“In accordance with predictions, significant differences in performance between knockouts and controls were observed (Paradee et al., 1999) (Fig.”
ResultCorrection of amygdalar dysfunction in a rat model of fragile X syndrome
Fernandes, G., Mishra, P. K., Nawaz, M. S., Donlin-Asp, P., Rahman, M. M., Hazra, A., Kedia, S., Kayenaat, A., Songara, D., Wyllie, D. J. A., et al. (2021) · Cell Reports · doi:10.1016/j.celrep.2021.109805 · Cited by 19
“At the other end, earlier studies on fearrelated behavior have yielded mixed results wherein knockout mice showed either impaired fear recall (Paradee et al., 1999) or no difference compared to normal mice (Dobkin et al., 2000; Hamilton et al., 2014; Peier et al., 2000).”
ResultHodges, S. L., Nolan, S. O., Tomac, L. A., Muhammad, I. D. A., Binder, M. S., Taube, J. H., & Lugo, J. N. (2019) · Physiology & Behavior · doi:10.1016/j.physbeh.2019.112776 · Cited by 20
Goddyn, H., Callaerts‐Vegh, Z., & D’Hooge, R. (2015) · The International Journal of Neuropsychopharmacology · doi:10.1093/ijnp/pyv053 · Cited by 18
Discrimination learning and attentional set formation in a mouse model of Fragile X.
Casten, K. S., Gray, A. C., & Burwell, R. D. (2011) · Behavioral Neuroscience · doi:10.1037/a0023561 · Cited by 22
“Fmr1 KO mice show subtle deficits in spatial learning and reversal learning (Consortium, 1994; D’Hooge et al., 1997; Paradee et al., 1999) and display impaired inhibitory control and attention in a five-choice serial reaction time task (Moon et al.”
BackgroundFragile X mental retardation protein in plasticity and disease
Todd, P. K., & Malter, J. S. (2002) · Journal of Neuroscience Research · doi:10.1002/jnr.10453 · Cited by 17
“First, FMR-1 knockout mice have normal long-term potentiation (LTP) in the hippocampus for at least 4 hr after induction (Godfraind et al., 1996; Paradee et al., 1999), although there is one report of a problem with cortical LTP (Li et al., 2002).”
BackgroundŚledziowska, M., Galloway, J., & Baudouin, S. J. (2019) · Neuroscience · doi:10.1016/j.neuroscience.2019.10.011 · Cited by 16
A Highly Selective MNK Inhibitor Rescues Deficits Associated with Fragile X Syndrome in Mice
Shukla, T. N., de la Peña, J. B., Perish, J. M., Ploski, J. E., Stumpf, C. R., Webster, K. R., Thorn, C. A., & Campbell, Z. T. (2020) · Neurotherapeutics · doi:10.1007/s13311-020-00932-4 · Cited by 19
Effects of regulatory BC1 RNA deletion on synaptic plasticity, learning, and memoryPreprint
Chung, A., Dahan, N., Alarcón, J. M., & Fenton, A. A. (2017) · Learning & Memory · doi:10.1101/lm.045617.117 · Cited by 16
“Phenotypic differences including fear-learning deficits due to Fmr1 deletion are weaker in C57Bl/6 mice compared with the 129×1/SvJ strain (Paradee et al. 1999; Moy et al. 2009; Spencer et al. 2011).”
BackgroundMoon, J., Ota, K. T., Driscoll, L. L., Levitsky, D. A., & Strupp, B. J. (2008) · Developmental Psychobiology · doi:10.1002/dev.20308 · Cited by 15
Longitudinal in vivo developmental changes of metabolites in the hippocampus of Fmr1 knockout mice
Shi, D., Xu, S., Waddell, J., Scafidi, S., Roys, S., Gullapalli, R. P., & McKenna, M. C. (2012) · Journal of Neurochemistry · doi:10.1111/jnc.12048 · Cited by 16
Kratovac, S., & Corbin, J. G. (2013) · Brain Research · doi:10.1016/j.brainres.2013.08.052 · Cited by 14
Synaptic Plasticity as a Therapeutic Target in the Treatment of Autism-related Single-gene Disorders
Pignatelli, M., Feligioni, M., Piccinin, S., Molinaro, G., Nicoletti, F., & Nisticò, R. (2013) · Current Pharmaceutical Design · doi:10.2174/1381612811319360008 · Cited by 14
“In fact, a number of studies have reported either impaired LTP [72,73] or normal LTP [74-76] in the CA1 region of the hippocampus.”
BackgroundPhenotypic changes in calbindin D28K immunoreactivity in the hippocampus of Fmr1 knockout mice
Real, M., Simón, M., Heredia, R., de Diego, Y. D., & Guirado, S. (2011) · The Journal of comparative neurology · doi:10.1002/cne.22643 · Cited by 15
“This model presents rather subtle differences in behavior and spatial learning compared to wildtype (WT) mice that are subject to strain background effects (Paradee et al., 1999; Dobkin et al., 2000; Van Dam et al., 2000).”
BackgroundSvalina, M. N., Rio, C. A. C.-D., Kushner, J. K., Levy, A., Baca, S. M., Guthman, E. M., Opendak, M. M., Sullivan, R. M., Restrepo, D., & Huntsman, M. M. (2022) · Journal of Neuroscience · doi:10.1523/jneurosci.1776-21.2022 · Cited by 13
Borreca, A., Luca, M. D., Ferrante, A., Boussadia, Z., Pignataro, A., Martire, A., & Ammassari‐Teule, M. (2023) · Scientific Reports · doi:10.1038/s41598-023-27991-9 · Cited by 14
Roy, R., Shiina, N., & Wang, D. O. (2019) · Neurobiology of Learning and Memory · doi:10.1016/j.nlm.2019.107149 · Cited by 14
Analysis of Fmr1 Deletion in a Subpopulation of Post‐Mitotic Neurons in Mouse Cortex and Hippocampus
Amiri, A., Sanchez‐Ortiz, E., Cho, W., Birnbaum, S. G., Xu, J., McKay, R. M., & Parada, L. F. (2014) · Autism Research · doi:10.1002/aur.1342 · Cited by 14
“In contrast to human patients, however, Fmr1 KO mice show reduced anxiety [Bakker et al. 1994; Paradee et al. 1999; Peier et al. 2000].”
BackgroundResultPietropaolo, S., & Subashi, E. (2014) · Cambridge University Press eBooks · doi:10.1017/cbo9781107360556.014 · Cited by 12
The development of cortical columns: role of Fragile X mental retardation protein
Bureau, I. (2009) · The Journal of Physiology · doi:10.1113/jphysiol.2008.167155 · Cited by 11
Luque, M. A., Morcuende, S., Torres, B., & Herrero, L. (2024) · The Journal of Physiology · doi:10.1113/jp285244 · Cited by 9
“The present study was carried out in mice with an FVB genetic background, instead of the C57BL/6 strain, and the opposite results have been explained by strain-dependent differences (Paradee et al. 1999; Routh et al., 2013).”
Behavioral, neurotransmitter and transcriptomic analyses in male and female Fmr1 KO mice
McCarthy, D. M., Vied, C., Trupiano, M. X., Canekeratne, A. J., Wang, Y., Schatschneider, C. W., & Bhide, P. G. (2024) · Frontiers in Behavioral Neuroscience · doi:10.3389/fnbeh.2024.1458502 · Cited by 10
Mouse model of fragile X syndrome: Behavioral and hormonal response to stressors.
Nielsen, D. M., Evans, J. J., Derber, W. J., Johnston, K. A., Laudenslager, M. L., Crnic, L. S., & Maclean, K. N. (2009) · Behavioral Neuroscience · doi:10.1037/a0015242 · Cited by 10
The mGluR Theory of Fragile X: From Mice to Men
Stoppel, L. J., Osterweil, E. K., & Bear, M. F. (2017) · Elsevier eBooks · doi:10.1016/b978-0-12-804461-2.00009-3 · Cited by 9
Effects of a social stimulus on gene expression in a mouse model of fragile X syndrome
Rogers, T. D., Anacker, A. M. J., Kerr, T. M., Forsberg, C. G., Wang, J., Zhang, B., & Veenstra-VanderWeele, J. M. (2017) · Molecular Autism · doi:10.1186/s13229-017-0148-6 · Cited by 11
Svalina, M. N., Guthman, E. M., Rio, C. A. C.-D., Kushner, J. K., Baca, S. M., Restrepo, D., & Huntsman, M. M. (2021) · eNeuro · doi:10.1523/eneuro.0113-21.2021 · Cited by 12
Genetics and Genomics of Neurobehavioral Disorders
Fisch, G. S. (2003) · Humana Press eBooks · doi:10.1385/1592593534 · Cited by 9
Dwivedi, D., Chattarji, S., & Bhalla, U. S. (2019) · eNeuro · doi:10.1523/eneuro.0217-19.2019 · Cited by 9
“Strain-dependent differences in FXS have previously been found (Paradee et al., 1999; Dobkin et al., 2000; Lee et al., 2011b; Routh et al., 2013).”
BackgroundDaems, C., Sekulić, M., Vulsteke, V., Loo, G., D’Hooge, R., Callaerts‐Vegh, Z., & Callaerts, P. (2019) · Brain Behavior & Immunity - Health · doi:10.1016/j.bbih.2019.100018 · Cited by 10
Animal Models of Fragile X Syndrome
Kooy, R. F., Jin, P., Bao, H., Till, S. M., Kind, P. C., & Willemsen, R. (2017) · Elsevier eBooks · doi:10.1016/b978-0-12-804461-2.00007-x · Cited by 8
Cage effects on synaptic plasticity and its modulation in a mouse model of fragile X syndrome
Volianskis, R., Lundbye, C. J., Petroff, G. N., Jane, D. E., Georgiou, J., & Collingridge, G. L. (2024) · Philosophical Transactions of the Royal Society B Biological Sciences · doi:10.1098/rstb.2023.0484 · Cited by 6
“…as poor maternal care [86] or exposure to social defeat [97], can influence memory in CFC. Studies examining CFC in Fmr1 KO mice have yielded diverse results, with some studies reporting deficits in contextual fear memory [39,54,70–72], while other studies found normal levels of freezing [73–77].”
ResultEmotion Potentiated Startle in Fragile X Syndrome
Ballinger, E. C., Cordeiro, L., Chavez, A. D., Hagerman, R. J., & Hessl, D. R. (2014) · Journal of Autism and Developmental Disorders · doi:10.1007/s10803-014-2125-7 · Cited by 7
“This is in keeping with work in the mouse model of FXS, which has demonstrated blunted long term potentiation in the amygdala and impaired fear potentiated startle, the homologous behavioral assay in rodents (Paradee et al. 1999; Suvrathan et al. 2010; Zhao 2005; Olmos-Serrano et al. 2010).”
From Discovery to Innovative Translational Approaches in 80 Years of Fragile X Syndrome Research
van der Lei, M. B., & Kooy, R. F. (2025) · Biomedicines · doi:10.3390/biomedicines13040805 · Cited by 6
“Over the past 30 years, various behavioral tests have demonstrated that Fmr1 KO mice display mild cognitive impairments in tasks like the Morris water maze, although results vary depending on genetic background [12,86–88].”
From circuits to behavior: Amygdala dysfunction in fragile X syndrome
Svalina, M. N., Sullivan, R. M., Restrepo, D., & Huntsman, M. M. (2023) · Frontiers in Integrative Neuroscience · doi:10.3389/fnint.2023.1128529 · Cited by 6
“However, studies employing classic Pavlovian fear-conditioning paradigms have identified decreases in contextual and conditioned freezing episodes suggesting deficiencies in both hippocampal and amygdala-based associative learning (Paradee et al., 1999).”
BackgroundAnimal Models of Fragile X Syndrome: Mice and Flies
Oostra, B. A., & Nelson, D. L. (2006) · Elsevier eBooks · doi:10.1016/b978-012369462-1/50012-0 · Cited by 6
Mouse Models of Hereditary Mental Retardation
Welzl, H., D’Adamo, P., Wolfer, D. P., & Lipp, H. (2006) · Contemporary clinical neuroscience · doi:10.1007/978-1-59745-058-4_6 · Cited by 6
The inhibition of NCS-1 binding to Ric8a rescues fragile X syndrome mice model phenotypes
Cogram, P., Fernández-Beltrán, L. C., Casarejos, M. J., Sánchez-Yepes, S., Rodríguez‐Martín, E., García‐Rubia, A., Sánchez-Barrena, M. J., Gil, C., Martı́nez, A., & Mansilla, A. (2022) · Frontiers in Neuroscience · doi:10.3389/fnins.2022.1007531 · Cited by 6
“Magnetic resonance imaging (MRI) studies have observed multiple differences in the neuroanatomy of male Fmr1 KO mice on a FVB but not in the B6 background (Paradee et al., 1999; Lai et al., 2016).”
BackgroundLau, B. W.-M., Yau, S., Po, K.-T., & So, K. (2016) · Elsevier eBooks · doi:10.1016/b978-0-12-801977-1.00011-8 · Cited by 5
Impaired spatial processing in a mouse model of fragile X syndrome
Ghilan, M., Bettio, L. E. B., Noonan, A., Brocardo, P. S., Gil‐Mohapel, J., & Christie, B. R. (2018) · Behavioural Brain Research · doi:10.1016/j.bbr.2018.05.012 · Cited by 3
“Indeed, the reported deficits shown by Fmr1 mice in the MWM test were subtle and evidence suggests they may be due to strain effects rather than the loss of FMRP [45].”
InfluentialGroß, C., & Bhattacharya, A. (2017) · Elsevier eBooks · doi:10.1016/b978-0-12-804461-2.00011-1 · Cited by 3
Fragile X-Linked Mental Retardation
Gantois, I., Kooy, R. F., & Oostra, B. A. (2006) · Wiley-VCH eBooks · doi:10.1002/3527600906.mcb.200300051 · Cited by 3
9th International workshop on Fragile X syndrome and X-linked mental retardation
Fryns, J., Borghgraef, M., Brown, T. W., Chelly, J., Fisch, G. S., Hamel, B. C. J., Hanauer, A., Lacombe, D., Luo, L., Macpherson, J., et al. (2000) · American Journal of Medical Genetics · doi:10.1002/1096-8628(20001023)94:5 · Cited by 9
Blandin, K. J., Narvaiz, D. A., Sullens, D. G., Womble, P. D., Hodges, S. L., Binder, M. S., Faust, A. M., Nguyen, P. H., Pranske, Z. J., & Lugo, J. N. (2024) · Brain Sciences · doi:10.3390/brainsci14090892 · Cited by 2
“However, superimposing seizures on a mouse model of Fragile X Syndrome resulted in minor behavior deficits and no additional molecular changes.”
BackgroundExaggerated NMDA Receptor-Primed Metaplasticity via SK Channel Dysregulation in Fmr1 Knockout Mice
Nomura, T., Morton, C., Marshall, J. J., & Contractor, A. (2026) · Journal of Neuroscience · doi:10.1523/jneurosci.1653-25.2026 · Cited by 2
O’Leary, H., & Benke, T. A. (2017) · Elsevier eBooks · doi:10.1016/b978-0-12-804066-9.00074-2 · Cited by 2
Models of Human Behavior: Talking to the Animals
Fisch, G. S. (2009) · Springer eBooks · doi:10.1007/978-0-387-76727-7_5 · Cited by 2
Transgenic Mouse Models and Human Psychiatric Disease
Jonathan Flint, C. (2006) · Contemporary clinical neuroscience · doi:10.1007/978-1-59745-058-4_2 · Cited by 2
“Paradee and colleagues present evidence of the 129 genetic effects on tests of visuo– spatial orientation (51).”
BackgroundHyperexcitability and loss of feedforward inhibition in the Fmr1 KO lateral amygdalaPreprint
Guthman, E. M., Svalina, M. N., Rio, C. A. C.-D., Kushner, J. K., Baca, S. M., Restrepo, D., & Huntsman, M. M. (2020) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2020.04.21.053652 · Cited by 2
The quest for treatment of cognitive impairment: AMPA and mGlu5 receptor modulators
Sun, M. (2008) · Expert Opinion on Therapeutic Patents · doi:10.1517/13543776.18.9.999 · Cited by 1
“It has been proposed, therefore, [36] that the absence of mGluR5-mediated synaptic plasticity in the neocortex as observed in FMR1 knockout mice could contribute to the cognitive and learning deficits, whereas both protein-dependent and -independent LTP in the hippocampus are normal [60,61] .”
BackgroundInvestigating Therapeutic Strategies for Fragile X Syndrome in Mice
Michalon, A., & Lindemann, L. (2012) · BioValley monographs · doi:10.1159/000339190 · Cited by 1
Translational Neuroimaging for Drug Discovery and Development in Autism Spectrum Disorders
Badura, L. L., Saxe, M., Risterucci, C., Brammer, M. J., McTighe, S. M., Ring, R. H., & Smith, D. G. (2012) · Elsevier eBooks · doi:10.1016/b978-0-12-386945-6.00009-3 · Cited by 1
Szumlinski, K. K., Beltran, J., Doren, E. V., Chavez, C. L. J., Domingo-Gonzalez, R. D., Reyes, C. M., Ary, A. W., Lang, A., Guo, W., Worley, P. F., et al. (2023) · eNeuro · doi:10.1523/eneuro.0423-22.2023 · Cited by 1
“…(Tian et al., 2017) and certain strains of Fmr1 KO mice (Kooy et al., 1996; D’Hooge et al., 1997; April 2023, 10(4) ENEURO.0423-22.2023 eNeuro.org Paradee et al., 1999; Dobkin et al., 2000; Van Dam et al., 2000; Baker et al., 2010) exhibit deficits in spatial memory assessed under MWM…”
BackgroundInfluentialInvestigation Of The Molecular Mechanisms Of Synaptic Tagging And Capture
Park, J. W. (2014) · Scholarly Commons (University of Pennsylvania) · Cited by 1
Neuroanatomical Considerations Specific to the Study of Neurogenetics
Galaburda, A. M., & Schmitt, J. E. (2003) · Humana Press eBooks · doi:10.1007/978-1-59259-353-8_2 · Cited by 5
“Although the learning deficits in this mutant mouse would suggest that FMR1 plays a role in long-term potentiation (LTP), no differences compared to control mice were found when hippocampal slices were stimulated electrically (80,81).”
BackgroundCosta, R. M., Elgersma, Y., & Silva, A. J. (2003) · Humana Press eBooks · doi:10.1007/978-1-59259-353-8_3 · Cited by 3
Bui, T. A., Shatto, J., Cuppens, T., Droit, A., & Bolduc, F. V. (2021) · Frontiers in Psychiatry · doi:10.3389/fpsyt.2021.730987 · Cited by 1
“further, as individuals with FXS have been shown to have abnormal amygdala connectivity (105, 106), and FMR1 KO mice have been also documented to exhibit amygdala-related behavioral deficits (107, 108).”
BackgroundSynaptic modifications in hippocampal CA3 pyramidal cells in an Alzheimer's mouse model
Zhang, P. (2018) · eDiss (Georg-August-Universität Göttingen) · doi:10.53846/goediss-6752 · Cited by 1
The Fragile X Syndrome and the Fragile X Mutation
Hirst, M. C., & Fisch, G. S. (2003) · Humana Press eBooks · doi:10.1007/978-1-59259-353-8_14 · Cited by 1
Visual Spatial Learning and Memory in Fragile X Syndrome and fmr1 Knockout Mice
MacLeod, L. S. (2013) · uO Research (University of Ottawa) · doi:10.20381/ruor-6693
“…of the murine model of FXS focusing on replicating the spatial processing deficit observed in affected humans have been mixed (The Dutch-Belgian Fragile-X Consortium 1994; Kooy et al., 1996; D’Hooge et al., 1997; Paradee et al., 1999; Dobkin, et al., 2000; Peier et al., 2000; Mineur et al., 2002).”
BackgroundInfluentialDifferentiation of neural stem cells in fragile X syndrome
Tervonen, T. A. (2008) · Työväentutkimus Vuosikirja
Social Behavior in FMR1 KO Mice: a Model of Fragile X Syndrome
McNaughton, C. H. (2006) · eCommons (Cornell University)
Genome-Wide Expression Profiles of Amygdala and Hippocampus in Mice After Fear Conditioning
Zhao, Z. Y., & Hu, Y. (2010) · Advances in neurobiology · doi:10.1007/978-1-4419-7197-5_12
History Tends to Repeat: FMR-1 Silencing in Fragile X Syndrome
Haas, J. G. (2007) · Eukaryon
A Fragile Synapse: Changes at the Synapse in Fragile X Syndrome
Webber, A. J., & Christie, B. R. (2006) · Springer eBooks · doi:10.1007/978-0-387-32562-0_31
Fragile X Syndrome: Steps towards Therapy
Pop, A. (2013) · RePub (Erasmus University Rotterdam)
Social dysfunction and mental retardation
Pietropaolo, S., Sluyter, F., & Crusio, W. E. (2014) · Cambridge University Press eBooks · doi:10.1017/cbo9781107360556.010
CircuitandPlasticityDefectsintheDeveloping SomatosensoryCortexofFmr1Knock-OutMice
Shepherd, G. M. (2008)
Multipotent neural stem/progenitor cells as a model of genetic neuronal diseases
Kärkkäinen, V. (2012) · UEF eRepo (University of Eastern Finland)
Brain-DerivedNeurotrophicFactorRescuesSynaptic PlasticityinaMouseModelofFragileXSyndrome
Lauterborn, J. C. (2007)
Social Behavioral Moderators of Executive Function Tasks in Fragile X Syndrome
Miller, K. M. (2019) · Carolina Digital Repository (University of North Carolina at Chapel Hill) · doi:10.17615/h5ab-kn85
Casten, K. S., Gray, A. C., & Burwell, R. D. (2011)
Interactions between Hebbian and Homeostatic Synaptic Plasticity in Hippocampal Circuits.
Iliff, A. J. (2014) · Deep Blue (University of Michigan)
Oliver, & Robert, J. (2017) · UNM’s Digital Repository (University of New Mexico)
Phenotypic And Electrophysiologic Characterization of a Mouse Model of Fragile X Syndrome
Roy, S. (2012) · doi:10.21007/etd.cghs.2012.0268
“…TERM FLUID LICKING STUDY* Introduction Many children with FXS exhibit autistic behaviors and deficits in motor coordination including speech articulation deficits (Dobkin et al. 2000; Fiala et al. 2002; Koekkoek et al. 2005; Paradee et al. 1999; Yan, Asafo-Adjei, Arnold, Brown, & Bauchwitz 2004).”
BackgroundDenman, R. B. (2011) · Results and problems in cell differentiation · doi:10.1007/978-3-642-21649-7_19
“Like the recent finding that depending on electrophysiological conditions, LTP in the hippocampus is either not altered (Paradee et al. 1999; Li et al. 2002; Zhang et al. 2009), or decreased (Lauterborn et al.”
BackgroundIsolation housing exacerbates Alzheimer’s Disease phenotype in aged APP KI micePreprint
Laroy, M., Saito, T., Saido, T., D’Hooge, R., & der Jeugd, A. V. (2019) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/777524
“Context- and cue-dependent fear conditioning (CFC) is studied using an adapted protocol from Paradee et al. (1999). Electric foot shocks are applied to evoke a freezing response, which is acknowledged as a reliable measure of innate and conditioned fear in rodents (Paradee et al.”
BackgroundMethodInfluentialWhat Can the Study of Behavioral Phenotypes Teach Us About the Pathway from Genes to Behavior?
Flint, J. (2003) · Humana Press eBooks · doi:10.1007/978-1-59259-353-8_4
Crawley, J. N. (2006) · Wiley eBooks · doi:10.1002/9780470119051.refs
Ecker, C. (2013) · Autism and child psychopathology series · doi:10.1007/978-1-4614-8250-5_6
Budniok, S., Odent, P., Callaerts‐Vegh, Z., Bosmans, G., & D’Hooge, R. (2023) · Neuroreport · doi:10.1097/wnr.0000000000001938
Potential pharmacological treatment of fragile X syndrome during adulthood
Xu, Z., & Zhao, M. (2009) · Neuroscience Bulletin · doi:10.1007/s12264-009-0909-0
Oxytocin and Vasopressin in Autism and Genetic Syndromes
Levin‐Decanini, T., Francis, S. M., Sagar, A., Liu, W., Carter, C. S., & Jacob, S. (2015) · Contemporary clinical neuroscience · doi:10.1007/978-1-4939-2190-4_14
“By most accounts Fmr1 KO mice appear to have relatively normal behavior, but research has shown that the behavioral and cognitive deficits of the KO are actually quite subtle and parallel FXS patients (Paradee et al. 1999; D’Hooge et al. 1997; Peier et al. 2000; Berry-Kravis et al. 2002).”
BackgroundSalas, I. H. (2017) · VUBIR (Vrije Universiteit Brussel)
Involvement of mGluR5/Homer crosstalk disruption in the pathophysiology of Fragile X Syndrome
Aloisi, E. (2015) · theses.fr (ABES) · doi:10.70675/e628d840zb6adz4e63z9d1fz71559b23fe24
“However, first investigations of NMDAR-dependent forms of plasticity failed to reveal a deficit in Fmr1 KO mice (Godfraind et al., 1996; Huber et al., 2002; Paradee et al., 1999).”
BackgroundSynaptic modifications in hippocampal CA3 pyramidal cells in an Alzheimer's mouse model
Zhang, P. (2017) · theses.fr (ABES) · doi:10.70675/74cb83e0z6db8z4bb5z838dz6e1bb1c6275f
The role of intrinsic neuronal excitability for Prelimbic network function
Sa, R. D. (2019) · theses.fr (ABES) · doi:10.70675/8b11b402z0ae7z4587z8084z65b59ce7ba62
Felgerolle, C. (2019) · theses.fr (ABES) · doi:10.70675/fbd5cf69zed5az4a57zbfdez48e26a937a72
Metadata
- Authors
- Paradee, W., Melikian, H. E., Rasmussen, D. L., Kenneson, A., Conn, P. J., & Warren, S. T.
- Affiliations
- Howard Hughes Medical Institute (US)
- Emory University (US)
- Corresponding author
- Warren, S.T.
- Venue
- Neuroscience
- Publisher
- Elsevier BV
- Published
- 1999-09
- Volume
- 94
- Issue
- 1
- Pages
- 185-192
- ISSN
- 0306-4522 (ISSN-L)
- OpenAlex
- W2110871941
- Cited by
- 305
- References
- 51
- Citations by year
- 2026: 2 · 2025: 1 · 2024: 5 · 2023: 7 · 2022: 4 · 2021: 5 · 2020: 9 · 2019: 15 · 2018: 11 · 2017: 17 · 2016: 5 · 2015: 14 · 2014: 19 · 2013: 16 · 2012: 15
- FWCI
- 4.77
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Neuroscience (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (R37 HD20521, P01 HD35576, R37HD020521, P01HD035576)
- 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 › Memory and Neural Mechanisms
- Keywords
- fragile X syndrome, Fmr1 knockout mice, FMRP, amygdala, contextual fear, spatial learning, hippocampal long-term potentiation, strain differences, C57BL/6 mice, 129 mice, freezing behavior, translational regulation
- MeSH terms
- Amygdala, Animals, Conditioning, Psychological, Electrophysiology, Fear, Female, Fragile X Syndrome, Genotype, Hippocampus, Male, Mice, Inbred C57BL, Nerve Tissue Proteins, Phenotype, Spatial Behavior, RNA-Binding Proteins, Long-Term Potentiation, Mice, Knockout, Maze Learning, Mice, Fragile X Mental Retardation Protein, Fragile X Messenger Ribonucleoprotein 1
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