Cited by 305Genetics and Neurodevelopmental DisordersNeuroscience and Neuropharmacology ResearchMemory and Neural Mechanisms

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Fragile X mouse: strain effects of knockout phenotype and evidence suggesting deficient amygdala function

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

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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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BackgroundResult
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BackgroundResult
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BackgroundResult
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Background
Fragile X syndrome, the Fragile X related proteins, and animal models
Hoogeveen, A. T., Willemsen, R., & Oostra, B. A. (2002) · Microscopy Research and Technique · doi:10.1002/jemt.10064 · Cited by 36
“…have, indeed, a distinct profile of behavioral and cognitive deficits, several attempts were made to assess these functions in the FMR1 animal model (Bakker et al., 1994; Brown et al., 1997; D’Hooge et al., 1997; Fisch et al., 1999; Kooy et al., 1996; Paradee et al., 1999; Peier et al., 2000).”
Influential
Deficits in trace fear memory in a mouse model of the schizophrenia risk gene TCF4
Brzózka, M. M., & Rossner, M. J. (2012) · Behavioural Brain Research · doi:10.1016/j.bbr.2012.10.001 · Cited by 36
CPEB and translational control by cytoplasmic polyadenylation: impact on synaptic plasticity, learning, and memory
Huang, Y., Méndez, R., Fernández, M., & Richter, J. D. (2023) · Molecular Psychiatry · doi:10.1038/s41380-023-02088-x · Cited by 36
Fear-Specific Amygdala Function in Children and Adolescents on the Fragile X Spectrum: A Dosage Response of the FMR1 Gene
Kim, S.-Y., Burris, J., Bassal, F., Koldewyn, K., Chattarji, S., Tassone, F., Hessl, D. R., & Rivera, S. M. (2012) · Cerebral Cortex · doi:10.1093/cercor/bhs341 · Cited by 39
“For instance, amygdaladependent fear learning has been shown to be impaired in FMR1-KO mice (Paradee et al. 1999).”
Background
Fragile X syndrome and model organisms: identifying potential routes of therapeutic intervention
Bhogal, B., & Jongens, T. A. (2010) · Disease Models & Mechanisms · doi:10.1242/dmm.002006 · Cited by 36
GABAA–benzodiazepine receptor complex sensitivity in 5-HT1A receptor knockout mice on a 129/Sv background
Pattij, T., Groenink, L., Oosting, R. S., van der Gugten, J., Maes, R. A. A., & Olivier, B. (2002) · European Journal of Pharmacology · doi:10.1016/s0014-2999(02)01893-9 · Cited by 34
Reduced perineuronal net expression in Fmr1 KO mice auditory cortex and amygdala is linked to impaired fear-associated memory
Reinhard, S. M., Rais, M., Afroz, S., Hanania, Y., Pendi, K., Espinoza, K., Rosenthal, R., Binder, D. K., Ethell, I. M., & Razak, K. A. (2019) · Neurobiology of Learning and Memory · doi:10.1016/j.nlm.2019.107042 · Cited by 37
“While multiple groups have shown altered fear memory in Fmr1 KO mice, the mechanisms underlying the deficits remain unclear (de Diego-Otero et al., 2009; Dobkin et al., 2000; Eadie, Cushman, Kannangara, Fanselow, & Christie, 2012; Olmos-Serrano, Corbin, & Burns, 2011; Paradee et al., 1999; RomeroZerbo et al., 2009).”
Fragile X Syndrome: A Model of Gene-Brain-Behavior Relationships
Hagerman, R. J., & Hagerman, P. J. (2001) · Molecular Genetics and Metabolism · doi:10.1006/mgme.2001.3225 · Cited by 33
Pathogenesis of Börjeson-Forssman-Lehmann syndrome: Insights from PHF6 function
Jahani‐Asl, A., Cheng, C., Zhang, C., & Bonni, A. (2016) · Neurobiology of Disease · doi:10.1016/j.nbd.2016.09.011 · Cited by 37
Fragile X mental retardation protein regulates heterosynaptic plasticity in the hippocampusPreprint
Connor, S. A., Hoeffer, C. A., Klann, E., & Nguyen, P. (2011) · Learning & Memory · doi:10.1101/lm.2043811 · Cited by 32
“…increase in synaptic strength believed to underlie specific types of learning and memory (Bliss and Collingridge 1993; Godfraind et al. 1996; Paradee et al. 1999; Larson et al. 2005; Lauterborn et al. 2007). b-Adrenergic receptors (b-ARs) are G protein-coupled receptors that enhance…”
Background
Fragile X syndrome and the amygdala
Suvrathan, A., & Chattarji, S. (2011) · Current Opinion in Neurobiology · doi:10.1016/j.conb.2011.04.005 · Cited by 31
“This also limits our ability to interpret earlier behavioral studies on anxiety and fear in the Fmr1 KO mouse, which have yielded mixed results that are not always consistent with the enhanced emotional symptoms observed in the human disease [10,23,24 ,25,26].”
The role of ARID1B, a BAF chromatin remodeling complex subunit, in neural development and behavior
Moffat, J. J., Jung, E., Ka, M., Smith, A. L., Jeon, B. T., Santen, G. W. E., & Kim, W.-Y. (2018) · Progress in Neuro-Psychopharmacology and Biological Psychiatry · doi:10.1016/j.pnpbp.2018.08.021 · Cited by 31
Defective memory engram reactivation underlies impaired fear memory recall in Fragile X syndrome
Li, J., Jiang, R. Y., Arendt, K. L., Hsu, Y., Zhai, S. R., & Chen, L. (2020) · eLife · doi:10.7554/elife.61882 · Cited by 34
Fragile X syndrome: from gene discovery to therapy
Heulens, I. (2010) · Frontiers in bioscience · doi:10.2741/3785 · Cited by 32
Alterations of Amino Acids and Monoamine Metabolism inMale Fmr1 Knockout Mice: A Putative Animal Model ofthe Human Fragile X Mental Retardation Syndrome
Gruß, M., & Braun, K. A. (2001) · Neural Plasticity · doi:10.1155/np.2001.285 · Cited by 31
Changes in Sensitivity of Reward and Motor Behavior to Dopaminergic, Glutamatergic, and Cholinergic Drugs in a Mouse Model of Fragile X Syndrome
Fish, E. W., Krouse, M. C., Stringfield, S. J., DiBerto, J. F., Robinson, J. E., & Malanga, C. J. (2013) · PLoS ONE · doi:10.1371/journal.pone.0077896 · Cited by 29
“cognitive impairments [10-13], increased spontaneous motor”
Adenosine A2A receptor inhibition reduces synaptic and cognitive hippocampal alterations in Fmr1 KO mice
Ferrante, A., Boussadia, Z., Borreca, A., Mallozzi, C., Pedini, G., Pacini, L., Pèzzola, A., Armida, M., Vincenzi, F., Varani, K., et al. (2021) · Translational Psychiatry · doi:10.1038/s41398-021-01238-5 · Cited by 26
Modelling fragile X syndrome in the laboratory setting: A behavioral perspective
Melancia, F., & Trezza, V. (2018) · Behavioural Brain Research · doi:10.1016/j.bbr.2018.04.042 · Cited by 31
“when the location of the platform is changed) phases of the task [62,63], while other studies did not find differences between Fmr1-KO and WT mice [167,169].”
A presynaptic role for FMRP during protein synthesis–dependent long-term plasticity in AplysiaPreprint
Till, S. M., Li, H.-L., Miniaci, M. C., Kandel, E. R., & Choi, Y. (2010) · Learning & Memory · doi:10.1101/lm.1958811 · Cited by 30
“This result is consistent with findings from several studies that suggest some forms of hippocampal long-term potentiation are unaffected in Fmr1 knockout mice (Godfraind et al. 1996; Paradee et al. 1999; Li et al. 2002; Larson et al. 2005).”
Result
FMRP Modulates Activity-Dependent Spine Plasticity by Binding Cofilin1 mRNA and Regulating Localization and Local Translation
Feuge, J., Scharkowski, F., Michaelsen‐Preusse, K., & Körte, M. (2019) · Cerebral Cortex · doi:10.1093/cercor/bhz059 · Cited by 28
Cataloguing and Selection of mRNAs Localized to Dendrites in Neurons and Regulated by RNA-Binding Proteins in RNA Granules
Ohashi, R., & Shiina, N. (2020) · Biomolecules · doi:10.3390/biom10020167 · Cited by 33
“Spatial memory in the Morris water maze (MWM) in Fmr1 KO mice was also both impaired [217,218] and unaffected [213,219,220].”
Background
Mouse models of fragile X-related disorders
Willemsen, R., & Kooy, R. F. (2023) · Disease Models & Mechanisms · doi:10.1242/dmm.049485 · Cited by 30
Unaltered hormonal response to stress in a mouse model of fragile X syndrome
Qin, M., & Smith, C. B. (2008) · Psychoneuroendocrinology · doi:10.1016/j.psyneuen.2008.03.010 · Cited by 29
“Genetic background can clearly have effects on behavior (Crawley et al., 1997) and can influence the effects of the fmr 1K O on behavioral phenotype ( Paradee et al., 1999; Dobkin et al., 2000).”
Background
Normal Performance of Fmr1 Mice on a Touchscreen Delayed Nonmatching to Position Working Memory Task
Leach, P. T., Hayes, J., Pride, M., Silverman, J. L., & Crawley, J. N. (2016) · eneuro · doi:10.1523/eneuro.0143-15.2016 · Cited by 27
“2016 eNeuro.sfn.org cued, and/or trace-cued fear conditioning were reported by some groups (Paradee et al., 1999; Zhao et al., 2005; Auerbach et al., 2011; Ding et al., 2014), while other researchers failed to detect fear conditioning deficits (Dobkin et al., 2000; Peier et al., 2000; Van Dam et…”
BackgroundMethodResultInfluential
New insights into the molecular pathophysiology of fragile X syndrome and therapeutic perspectives from the animal model
Busquets-García, A., Maldonado, R. J., & Ozaita, A. (2014) · The International Journal of Biochemistry & Cell Biology · doi:10.1016/j.biocel.2014.05.004 · Cited by 28
“Other tests demonstrate mild alterations such as those on spatial learning and reversal learning assessed in the Morris or plus-shaped water maze test (D’Hooge et al., 1997; Van Dam et al., 2000), or those on associative learning (Hayashi et al., 2007; Michalon et al., 2012; Paradee et al., 1999).”
Influential
FMRP and CYFIP1 at the Synapse and Their Role in Psychiatric Vulnerability
Clifton, N. E., Thomas, K. L., Wilkinson, L. S., Hall, J., & Trent, S. (2020) · Complex Psychiatry · doi:10.1159/000506858 · Cited by 28
Effect of genetic background on acoustic startle response in fragile X knockout mice
Errijgers, V., Fransén, E., D’Hooge, R., Deyn, P. P. D., & Kooy, R. F. (2008) · Genetics Research · doi:10.1017/s0016672308009415 · Cited by 27
Inhibition of GluN2A NMDA receptors ameliorates synaptic plasticity deficits in the Fmr1−/y mouse model
Lundbye, C. J., Toft, A. K. H., & Banke, T. G. (2018) · The Journal of Physiology · doi:10.1113/jp276304 · Cited by 26
Olfactory discrimination learning in mice lacking the fragile X mental retardation protein
Larson, J., Kim, D., Patel, R. C., & Floreani, C. V. (2008) · Neurobiology of Learning and Memory · doi:10.1016/j.nlm.2008.01.002 · Cited by 26
“…mice have used aversively-motivated tasks such as water mazes (Bakker et al., 1994; D’Hooge et al., 1997; Dobkin et al., 2000; Kooy et al., 1996; Paradee et al., 1999; Peier et al., 2000; Van Dam et al., 2000); or fear conditioning/shock avoidance (Bakker et al., 1994; Brennan et al., 2006;…”
BackgroundInfluential
High‐throughput analysis of vocalizations reveals sex‐specific changes in Fmr1 mutant pups
Nolan, S. O., Hodges, S. L., & Lugo, J. N. (2019) · Genes Brain & Behavior · doi:10.1111/gbb.12611 · Cited by 25
“different background strains used.(20,21) In non-transgenic mouse lines,”
Background
Aberrant Neural Function During Emotion Attribution in Female Subjects With Fragile X Syndrome
Hagan, C. C., Hoeft, F., Mackey, A. P., Mobbs, D., & Reiss, A. L. (2008) · Journal of the American Academy of Child & Adolescent Psychiatry · doi:10.1097/chi.0b013e3181886e92 · Cited by 29
The Morris water maze.
Mulder, G. B., & Pritchett, K. R. (2003) · PubMed · Cited by 26
Parental origin impairment of synaptic functions and behaviors in cytoplasmic FMRP interacting protein 1 (Cyfip1) deficient mice
Chung, L., Wang, X., Zhu, L., Towers, A. J., Cao, X., Kim, I. H., & Jiang, Y. (2015) · Brain Research · doi:10.1016/j.brainres.2015.10.015 · Cited by 25
Preclinical testing of the ketogenic diet in fragile X mice
Westmark, P. R., Gutierrez, A., Gholston, A. K., Wilmer, T. M., & Westmark, C. J. (2020) · Neurochemistry International · doi:10.1016/j.neuint.2020.104687 · Cited by 27
“…Van Dam et al., 2000) as well as reports of decreased learning in the Fmr1KO (de Diego-Otero et al., 2009; R. M. Deacon et al., 2015; Mao et al., 2013; Martinez & Tejada-Simon, 2018; Neuwirth et al., 2015; Nolan et al., 2017; Olmos-Serrano et al, 2011; Paradee et al., 1999; Reinhard et al., 2019).”
Background
Amygdala Regulation of Fear and Emotionality in Fragile X Syndrome
Olmos-Serrano, J. L., & Corbin, J. G. (2011) · Developmental Neuroscience · doi:10.1159/000329424 · Cited by 25
Synaptic Plasticity in Mouse Models of Autism Spectrum Disorders
Chung, L., Bey, A. L., & Jiang, Y. (2012) · Korean Journal of Physiology and Pharmacology · doi:10.4196/kjpp.2012.16.6.369 · Cited by 27
“(only male used) CA1 L-LTP 3 trains of TBS (10 bursts) NS [38]”
Background
The Female and the Fragile X Reviewed
Kenneson, A., & Warren, S. T. (2001) · Seminars in Reproductive Medicine · doi:10.1055/s-2001-15401 · Cited by 25
Social behavior phenotypes in fragile X syndrome, autism, and the Fmr1 knockout mouse: Theoretical comment on McNaughton et al. (2008).
Brodkin, E. S. (2008) · Behavioral Neuroscience · doi:10.1037/0735-7044.122.2.483 · Cited by 24
“However, cognitive deficits in the Fmr1 knockout mouse have been surprisingly mild or not present and dependent on the mouse strain background (Dobkin et al., 2000; Frankland et al., 2004; Paradee et al., 1999).”
Fragile X (fmr1) mRNA expression is differentially regulated in two adult models of activity-dependent gene expression
Valentine, G. W., Chakravarty, S., Sarvey, J. M., Bramham, C. R., & Herkenham, M. A. (2000) · Molecular Brain Research · doi:10.1016/s0169-328x(99)00310-1 · Cited by 24
Adult Fmr1 knockout mice present with deficiencies in hippocampal interleukin-6 and tumor necrosis factor-α expression
Hodges, S. L., Nolan, S. O., Taube, J. H., & Lugo, J. N. (2017) · Neuroreport · doi:10.1097/wnr.0000000000000905 · Cited by 23
Single mild traumatic brain injury results in transiently impaired spatial long‐term memory and altered search strategies
Marschner, L., Schreurs, A., Lechat, B., Mogensen, J., Roebroek, A., Ahmed, T., & Balschun, D. (2019) · Behavioural Brain Research · doi:10.1016/j.bbr.2018.02.040 · Cited by 22
eIF4E/Fmr1 double mutant mice display cognitive impairment in addition to ASD-like behaviors
Huynh, T. N., Shah, M., Koo, S. Y., Faraud, K. S., Santini, E., & Klann, E. (2015) · Neurobiology of Disease · doi:10.1016/j.nbd.2015.08.016 · Cited by 24
“One limitation in studying the Fmr1 KO mouse is that strain differences can strongly influence phenotypes exhibited by these mice, leading to disparate outcomes (Dobkin et al., 2000; Paradee et al., 1999; Spencer et al., 2011).”
BackgroundResult
A comparative study of the performance of individuals with fragile X syndrome and Fmr1 knockout mice on Hebb‐Williams mazes
MacLeod, L. S., Kogan, C. S., Collin, C. A., Berry‐Kravis, E., Messier, C., & Gandhi, R. M. (2009) · Genes Brain & Behavior · doi:10.1111/j.1601-183x.2009.00534.x · Cited by 22
Insular volume reduction in fragile X syndrome
Cohen, J. D., Nichols, T. A., Brignone, L., Hall, S. S., & Reiss, A. L. (2011) · International Journal of Developmental Neuroscience · doi:10.1016/j.ijdevneu.2011.01.003 · Cited by 23
“All rights reserved. doi:10.1016/j.ijdevneu.2011.01.003 FraX phenotype as fmr1 knockout mice show an abnormal conditioned fear response (Paradee et al., 1999).”
Influential

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.
ORCID: Paradee, W.
Melikian, H.E.
Kenneson, A.
Conn, P.J.
Warren, S.T.
Venue: Neuroscience
Publisher: Elsevier BV
Published: 1999-09
Volume: 94
Issue: 1
Pages: 185-192
ISSN: 0306-4522 (ISSN-L)
DOI: 10.1016/s0306-4522(99)00285-7
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
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
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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Fragile X mouse: strain effects of knockout phenotype and evidence suggesting deficient amygdala function

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