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Volumn 100, Issue 1-4, 2003, Pages 111-123

Understanding fragile X syndrome: Insights from animal models

Author keywords

[No Author keywords available]

Indexed keywords

FRAGILE X MENTAL RETARDATION PROTEIN; RNA BINDING PROTEIN;

EID: 0344962385     PISSN: 14248581     EISSN: None     Source Type: Journal    
DOI: 10.1159/000072845     Document Type: Review
Times cited : (76)

References (169)
  • 1
    • 0027300283 scopus 로고
    • Nucleus basalis magnocellularis and hippocampus are the major sites of FMR-1 expression in the human fetal brain
    • 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 Genet 4:147-153 (1993).
    • (1993) Nature Genet , vol.4 , pp. 147-153
    • Abitbol, M.1    Menini, C.2    Delezoide, A.L.3    Rhyner, T.4    Vekemans, M.5    Mallet, J.6
  • 2
    • 0032847774 scopus 로고    scopus 로고
    • Dissecting FMR1, the protein responsible for fragile X syndrome, in its structural and functional domains
    • Adinolfi S, Bagni C, Musco G, Gibson T, Mazzarella L, Pastore A: Dissecting FMR1, the protein responsible for fragile X syndrome, in its structural and functional domains. RNA 5:1248-1258 (1999).
    • (1999) RNA , vol.5 , pp. 1248-1258
    • Adinolfi, S.1    Bagni, C.2    Musco, G.3    Gibson, T.4    Mazzarella, L.5    Pastore, A.6
  • 3
    • 0026008176 scopus 로고
    • Novel form of long-term potentiation produced by a K+ channel blocker in the hippocampus
    • Aniksztejn L, Ben-Ari Y: Novel form of long-term potentiation produced by a K+ channel blocker in the hippocampus. Nature 349:67-69 (1991).
    • (1991) Nature , vol.349 , pp. 67-69
    • Aniksztejn, L.1    Ben-Ari, Y.2
  • 4
    • 0027377580 scopus 로고
    • FMR1 protein: Conserved RNP family domains and selective RNA binding
    • Ashley C Jr, Wilkinson KD, Reines D, Warren ST: FMR1 protein: conserved RNP family domains and selective RNA binding. Science 262:563-568 (1993a).
    • (1993) Science , vol.262 , pp. 563-568
    • Ashley Jr., C.1    Wilkinson, K.D.2    Reines, D.3    Warren, S.T.4
  • 6
    • 0030078594 scopus 로고    scopus 로고
    • Ionotropic glutamate receptors: Their possible role in the expression of hippocampal synaptic plasticity
    • Asztely F, Gustafsson B: Ionotropic glutamate receptors: Their possible role in the expression of hippocampal synaptic plasticity. Mol Neurobiol 12: 1-11 (1996).
    • (1996) Mol Neurobiol , vol.12 , pp. 1-11
    • Asztely, F.1    Gustafsson, B.2
  • 7
    • 0029013598 scopus 로고
    • Impairment of spatial but not contextual memory in CaMKII mutant mice with a selective loss of hippocampal LTP in the range of the theta frequency
    • Bach ME, Hawkins RD, Osman M, Kandel ER, Mayford M: Impairment of spatial but not contextual memory in CaMKII mutant mice with a selective loss of hippocampal LTP in the range of the theta frequency. Cell 81:905-915 (1995).
    • (1995) Cell , vol.81 , pp. 905-915
    • Bach, M.E.1    Hawkins, R.D.2    Osman, M.3    Kandel, E.R.4    Mayford, M.5
  • 8
    • 0027715424 scopus 로고
    • Enhanced expression of the murine FMR1 gene during germ cell proliferation suggests a special function in both the male and the female gonad
    • Bächner D, Manca A, Steinbach P, Wöhrle D, Just W, Vogel W, Hameister H, Poustka A: Enhanced expression of the murine FMR1 gene during germ cell proliferation suggests a special function in both the male and the female gonad. Hum molec Genet 2:2043-2050 (1993).
    • (1993) Hum Molec Genet , vol.2 , pp. 2043-2050
    • Bächner, D.1    Manca, A.2    Steinbach, P.3    Wöhrle, D.4    Just, W.5    Vogel, W.6    Hameister, H.7    Poustka, A.8
  • 12
    • 0026601010 scopus 로고
    • Cyclic AMP metabolism in fragile X syndrome
    • Berry-Kravis E, Huttenlocher PR: Cyclic AMP metabolism in fragile X syndrome. Ann Neurol 31:22-26 (1992).
    • (1992) Ann Neurol , vol.31 , pp. 22-26
    • Berry-Kravis, E.1    Huttenlocher, P.R.2
  • 13
    • 0027476024 scopus 로고
    • A synaptic model of memory: Long-term potentiation in the hippocampus
    • Bliss TV, Collingridge GL: A synaptic model of memory: long-term potentiation in the hippocampus. Nature 361:31-39 (1993).
    • (1993) Nature , vol.361 , pp. 31-39
    • Bliss, T.V.1    Collingridge, G.L.2
  • 18
    • 0032546976 scopus 로고    scopus 로고
    • Purified recombinant Fmrp exhibits selective RNA binding as an intrinsic property of the fragile X mental retardation protein
    • Brown V, Small K, Lakkis L, Feng Y, Gunter C, Wilkinson KD, Warren ST: Purified recombinant Fmrp exhibits selective RNA binding as an intrinsic property of the fragile X mental retardation protein. J biol Chem 273:15521-15527 (1998).
    • (1998) J Biol Chem , vol.273 , pp. 15521-15527
    • Brown, V.1    Small, K.2    Lakkis, L.3    Feng, Y.4    Gunter, C.5    Wilkinson, K.D.6    Warren, S.T.7
  • 20
    • 0035804765 scopus 로고    scopus 로고
    • Fragile X mice develop sensory hyper-reactivity to auditory stimuli
    • Chen L, Toth M: Fragile X mice develop sensory hyper-reactivity to auditory stimuli. Neuroscience 103: 1043-1050 (2001).
    • (2001) Neuroscience , vol.103 , pp. 1043-1050
    • Chen, L.1    Toth, M.2
  • 21
    • 0032741429 scopus 로고    scopus 로고
    • Synergistic effect of histone hyperacetylation and DNA demethylation in the reactivation of the FMR1 gene
    • Chiurazzi P, Pomponi MG, Pietrobono R, Bakker CE, Neri G, Oostra BA: Synergistic effect of histone hyperacetylation and DNA demethylation in the reactivation of the FMR1 gene. Hum molec Genet 8:2317-2323 (1999).
    • (1999) Hum Molec Genet , vol.8 , pp. 2317-2323
    • Chiurazzi, P.1    Pomponi, M.G.2    Pietrobono, R.3    Bakker, C.E.4    Neri, G.5    Oostra, B.A.6
  • 23
    • 0026069511 scopus 로고
    • Effects of age and communication level on eye contact in fragile X males and non-fragile X autistic males
    • Cohen IL, Vietze PM, Sudhalter V, Jenkins EC, Brown WT: Effects of age and communication level on eye contact in fragile X males and non-fragile X autistic males. Am J med Genet 38:498-502 (1991).
    • (1991) Am J Med Genet , vol.38 , pp. 498-502
    • Cohen, I.L.1    Vietze, P.M.2    Sudhalter, V.3    Jenkins, E.C.4    Brown, W.T.5
  • 25
    • 0030760613 scopus 로고    scopus 로고
    • The fragile X mental retardation protein is associated with poly(A) (+) mRNA in actively translating polyribosomes
    • Corbin F, Bouillon M, Fortin A, Morin S, Rousseau F, Khandjian EW: The fragile X mental retardation protein is associated with poly(A) (+) mRNA in actively translating polyribosomes. Hum molec Genet 6:1465-1472 (1997).
    • (1997) Hum Molec Genet , vol.6 , pp. 1465-1472
    • Corbin, F.1    Bouillon, M.2    Fortin, A.3    Morin, S.4    Rousseau, F.5    Khandjian, E.W.6
  • 26
    • 0023631723 scopus 로고
    • Hippocampal mossy fiber distribution co-varies with open-field habituation in the mouse
    • Crusio WE, Schwegler H: Hippocampal mossy fiber distribution co-varies with open-field habituation in the mouse. Behav Brain Res 26:153-158 (1987).
    • (1987) Behav Brain Res , vol.26 , pp. 153-158
    • Crusio, W.E.1    Schwegler, H.2
  • 27
    • 0029998871 scopus 로고    scopus 로고
    • The dynamics of dendritic structure in developing hippocampal slices
    • Dailey ME, Smith SJ: The dynamics of dendritic structure in developing hippocampal slices. J Neurosci 16:2983-2994 (1996).
    • (1996) J Neurosci , vol.16 , pp. 2983-2994
    • Dailey, M.E.1    Smith, S.J.2
  • 28
    • 0028821928 scopus 로고
    • Modulation of learning processes by ionotropic glutamate receptor ligands
    • Danysz W, Zajaczkowski W, Parsons CG: Modulation of learning processes by ionotropic glutamate receptor ligands. Behav Pharmacol 6:455-474 (1995).
    • (1995) Behav Pharmacol , vol.6 , pp. 455-474
    • Danysz, W.1    Zajaczkowski, W.2    Parsons, C.G.3
  • 29
    • 0035900649 scopus 로고    scopus 로고
    • Fragile X mental retardation protein targets G quartet mRNAs important for neuronal function
    • Darnell JC, Jensen KB, Jin P, Brown V, Warren ST, Darnell RB: Fragile X mental retardation protein targets G quartet mRNAs important for neuronal function. Cell 107:489-499 (2001).
    • (2001) Cell , vol.107 , pp. 489-499
    • Darnell, J.C.1    Jensen, K.B.2    Jin, P.3    Brown, V.4    Warren, S.T.5    Darnell, R.B.6
  • 38
    • 0027176361 scopus 로고
    • The FMR-1 protein is cytoplasmic, most abundant in neurons and appears normal in carriers of a fragile X premutation
    • 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 Genet 4:335-340 (1993).
    • (1993) Nature Genet , vol.4 , pp. 335-340
    • Devys, D.1    Lutz, Y.2    Rouyer, N.3    Bellocq, J.P.4    Mandel, J.L.5
  • 42
    • 0029816723 scopus 로고    scopus 로고
    • The fragile X mental retardation protein is a ribosonucleo-protein containing both nuclear localization and nuclear export signals
    • Eberhart DE, Malter HE, Feng Y, Warren ST: The fragile X mental retardation protein is a ribosonucleo-protein containing both nuclear localization and nuclear export signals. Hum molec Genet 5:1083-1091 (1996).
    • (1996) Hum Molec Genet , vol.5 , pp. 1083-1091
    • Eberhart, D.E.1    Malter, H.E.2    Feng, Y.3    Warren, S.T.4
  • 43
    • 0031310667 scopus 로고    scopus 로고
    • FMRP associates with polyribosomes as an mRNP, and the I 304N mutation of severe fragile X syndrome abolishes this association
    • 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. Mol Cell 1:109-118 (1997).
    • (1997) Mol Cell , vol.1 , pp. 109-118
    • Feng, Y.1    Absher, D.2    Eberhart, D.E.3    Brown, V.4    Malter, H.E.5    Warren, S.T.6
  • 44
    • 0028979161 scopus 로고
    • Quantitative comparison of FMR1 gene expression in normal and premutation alleles
    • Feng Y, Lakkis D, Warren ST: Quantitative comparison of FMR1 gene expression in normal and premutation alleles. Am J hum Genet 56:106-113 (1995a).
    • (1995) Am J Hum Genet , vol.56 , pp. 106-113
    • Feng, Y.1    Lakkis, D.2    Warren, S.T.3
  • 46
    • 0026653163 scopus 로고
    • Is autism associated with the fragile X syndrome?
    • Fisch GS: Is autism associated with the fragile X syndrome? Am J med Genet 43:78-86 (1992).
    • (1992) Am J Med Genet , vol.43 , pp. 78-86
    • Fisch, G.S.1
  • 47
    • 0027160981 scopus 로고
    • What is associated with the fragile X syndrome?
    • Fisch GS: What is associated with the fragile X syndrome?. Am J med Genet 48:112-121 (1993).
    • (1993) Am J Med Genet , vol.48 , pp. 112-121
    • Fisch, G.S.1
  • 48
  • 49
    • 0035217080 scopus 로고    scopus 로고
    • Apoptosis in yeast: A new model for aging research
    • Frohlich KU, Madeo F: Apoptosis in yeast: a new model for aging research. Exp Gerontol 37:27-31 (2001).
    • (2001) Exp Gerontol , vol.37 , pp. 27-31
    • Frohlich, K.U.1    Madeo, F.2
  • 54
    • 0027092647 scopus 로고
    • Impaired long-term potentiation, spatial learning, and hippocampal development in fyn mutant mice
    • Grant SG, O'Dell TJ, Karl KA, Stein PL, Soriano P, Kandel ER: Impaired long-term potentiation, spatial learning, and hippocampal development in fyn mutant mice. Science 258:1903-1910 (1992).
    • (1992) Science , vol.258 , pp. 1903-1910
    • Grant, S.G.1    O'Dell, T.J.2    Karl, K.A.3    Stein, P.L.4    Soriano, P.5    Kandel, E.R.6
  • 57
    • 0001966753 scopus 로고    scopus 로고
    • Physical and behavioral phenotype
    • Hagerman RJ, Cronister A (eds), The Johns Hopkins University Press, Baltimore
    • Hagerman R: Physical and behavioral phenotype, in Hagerman RJ, Cronister A (eds): Fragile-X Syndrome: Diagnosis, Treatment and Research, pp 3-87 (The Johns Hopkins University Press, Baltimore 1996).
    • (1996) Fragile-X Syndrome: Diagnosis, Treatment and Research , pp. 3-87
    • Hagerman, R.1
  • 58
    • 0001966753 scopus 로고    scopus 로고
    • The physical and behavioral phenotype
    • Hagerman RJ, Hagerman PJ (eds), The Johns Hopkins University Press, Baltimore
    • Hagerman RJ: The physical and behavioral phenotype, in Hagerman RJ, Hagerman PJ (eds): Fragile-X Syndrome: Diagnosis, Treatment and Research, pp 3-109 (The Johns Hopkins University Press, Baltimore 2002).
    • (2002) Fragile-X Syndrome: Diagnosis, Treatment and Research , pp. 3-109
    • Hagerman, R.J.1
  • 63
  • 65
    • 0027377155 scopus 로고
    • High resolution methylation analysis of the FMR1 gene trinucleotide repeat region in fragile X syndrome
    • Hornstra IK, Nelson DL, Warren ST, Yang TP High resolution methylation analysis of the FMR1 gene trinucleotide repeat region in fragile X syndrome. Hum molec Genet 2:1659-1665 (1993).
    • (1993) Hum Molec Genet , vol.2 , pp. 1659-1665
    • Hornstra, I.K.1    Nelson, D.L.2    Warren, S.T.3    Yang, T.P.4
  • 66
    • 0037188502 scopus 로고    scopus 로고
    • Altered synaptic plasticity in a mouse model of fragile X mental retardation
    • Huber KM, Gallagher SM, Warren ST, Bear MF: Altered synaptic plasticity in a mouse model of fragile X mental retardation. Proc natl Acad Sci, USA 99:7746-7750 (2002).
    • (2002) Proc Natl Acad Sci, USA , vol.99 , pp. 7746-7750
    • Huber, K.M.1    Gallagher, S.M.2    Warren, S.T.3    Bear, M.F.4
  • 67
    • 0034685813 scopus 로고    scopus 로고
    • Role for rapid dendritic protein synthesis in hippocampal mGluR-dependent long-term depression
    • Huber KM, Kayser MS, Bear MF: Role for rapid dendritic protein synthesis in hippocampal mGluR-dependent long-term depression. Science 288: 1254-1257 (2000).
    • (2000) Science , vol.288 , pp. 1254-1257
    • Huber, K.M.1    Kayser, M.S.2    Bear, M.F.3
  • 68
    • 0034942216 scopus 로고    scopus 로고
    • Chemical induction of mGluR5- and protein synthesis-dependent long-term depression in hippocampal area CA1
    • Huber KM, Roder JC, Bear MF: Chemical induction of mGluR5- and protein synthesis-dependent long-term depression in hippocampal area CA1. J Neurophysiol 86:321-325 (2001).
    • (2001) J Neurophysiol , vol.86 , pp. 321-325
    • Huber, K.M.1    Roder, J.C.2    Bear, M.F.3
  • 71
    • 0036197027 scopus 로고    scopus 로고
    • Altered mossy fiber distributions in adult Fmr1 (FVB), knockout mice
    • Ivanco TL, Greenough WT: Altered mossy fiber distributions in adult Fmr1 (FVB). knockout mice. Hippocampus 12:47-54 (2002).
    • (2002) Hippocampus , vol.12 , pp. 47-54
    • Ivanco, T.L.1    Greenough, W.T.2
  • 73
    • 0034639857 scopus 로고    scopus 로고
    • Understanding the molecular basis of fragile X syndrome
    • Jin P, Warren ST: Understanding the molecular basis of fragile X syndrome. Hum molec Genet 9:901-908 (2000).
    • (2000) Hum Molec Genet , vol.9 , pp. 901-908
    • Jin, P.1    Warren, S.T.2
  • 74
    • 0030059545 scopus 로고    scopus 로고
    • The fragile X mental retardation protein is associated with ribosomes
    • Khandjian E, Corbin F, Woerly S, Rousseau F: The fragile X mental retardation protein is associated with ribosomes. Nature Genet 12:91-93 (1996).
    • (1996) Nature Genet , vol.12 , pp. 91-93
    • Khandjian, E.1    Corbin, F.2    Woerly, S.3    Rousseau, F.4
  • 75
    • 0035885862 scopus 로고    scopus 로고
    • Comparative genomic sequence analysis of the FXR gene family: FMR1, FXR 1, and FXR2
    • Kirkpatrick LL, McIlwain KA, Nelson DL: Comparative genomic sequence analysis of the FXR gene family: FMR1, FXR1, and FXR2. Genomics 78: 169-177 (2001).
    • (2001) Genomics , vol.78 , pp. 169-177
    • Kirkpatrick, L.L.1    McIlwain, K.A.2    Nelson, D.L.3
  • 79
    • 0031054284 scopus 로고    scopus 로고
    • Follicle stimulating hormone is required for ovarian follicle maturation but not male fertility
    • Kumar TR, Wang Y, Lu N, Matzuk MM: Follicle stimulating hormone is required for ovarian follicle maturation but not male fertility. Nature Genet 15:201-204 (1997).
    • (1997) Nature Genet , vol.15 , pp. 201-204
    • Kumar, T.R.1    Wang, Y.2    Lu, N.3    Matzuk, M.M.4
  • 82
    • 0032104278 scopus 로고    scopus 로고
    • Long uninterrupted CGG repeats within the first exon of the human FMR1 gene are not intrinsically unstable in transgenic mice
    • Lavedan C, Grabczyk E, Usdin K, Nussbaum RL: Long uninterrupted CGG repeats within the first exon of the human FMR1 gene are not intrinsically unstable in transgenic mice. Genomics 50:229-240 (1998).
    • (1998) Genomics , vol.50 , pp. 229-240
    • Lavedan, C.1    Grabczyk, E.2    Usdin, K.3    Nussbaum, R.L.4
  • 83
    • 0030931869 scopus 로고    scopus 로고
    • 21 from the fragile X gene remain stable in transgenic mice
    • 21 from the fragile X gene remain stable in transgenic mice. Hum Genet 100:407-414 (1997).
    • (1997) Hum Genet , vol.100 , pp. 407-414
    • Lavedan, C.N.1    Garrett, L.2    Nussbaum, R.L.3
  • 84
    • 0036198673 scopus 로고    scopus 로고
    • Reduced cortical synaptic plasticity and GluR1 expression associated with fragile X mental retardation protein deficiency
    • Li J, Pelletier MR, Perez Velazquez JL, Carlen PL: Reduced cortical synaptic plasticity and GluR1 expression associated with fragile X mental retardation protein deficiency. Mol Cell Neurosci 19: 138-151 (2002).
    • (2002) Mol Cell Neurosci , vol.19 , pp. 138-151
    • Li, J.1    Pelletier, M.R.2    Perez Velazquez, J.L.3    Carlen, P.L.4
  • 85
    • 0035368955 scopus 로고    scopus 로고
    • The fragile X mental retardation protein inhibits translation via interacting with mRNA
    • Li Z, Zhang Y, Ku L, Wilkinson KD, Warren ST, Feng Y: The fragile X mental retardation protein inhibits translation via interacting with mRNA. Nucl Acids Res 29:2276-2283 (2001).
    • (2001) Nucl Acids Res , vol.29 , pp. 2276-2283
    • Li, Z.1    Zhang, Y.2    Ku, L.3    Wilkinson, K.D.4    Warren, S.T.5    Feng, Y.6
  • 86
    • 0035132869 scopus 로고    scopus 로고
    • Activation of synaptic NMDA receptors induces membrane insertion of new AMPA receptors and LTP in cultured hippocampal neurons
    • Lu W, Man H, Ju W, Trimble WS, MacDonald JF, Wang YT: Activation of synaptic NMDA receptors induces membrane insertion of new AMPA receptors and LTP in cultured hippocampal neurons. Neuron 29:243-254 (2001).
    • (2001) Neuron , vol.29 , pp. 243-254
    • Lu, W.1    Man, H.2    Ju, W.3    Trimble, W.S.4    MacDonald, J.F.5    Wang, Y.T.6
  • 87
    • 0028295792 scopus 로고
    • Cognitive functioning and information processing of adult mentally retarded men with fragile-X syndrome
    • Maes B, Fryns JP, Van Walleghem M, Van den Berghe H: Cognitive functioning and information processing of adult mentally retarded men with fragile-X syndrome. Am J med Genet 50:190-200 (1994).
    • (1994) Am J Med Genet , vol.50 , pp. 190-200
    • Maes, B.1    Fryns, J.P.2    Van Walleghem, M.3    Van Den Berghe, H.4
  • 90
    • 0030475008 scopus 로고    scopus 로고
    • Impaired hippocampal representation of space in CA1-specific NMDAR1 knockout mice
    • McHugh TJ, Blum KI, Tsien JZ, Tonegawa S, Wilson MA: Impaired hippocampal representation of space in CA1-specific NMDAR1 knockout mice. Cell 87:1339-1349 (1996).
    • (1996) Cell , vol.87 , pp. 1339-1349
    • McHugh, T.J.1    Blum, K.I.2    Tsien, J.Z.3    Tonegawa, S.4    Wilson, M.A.5
  • 93
    • 0031813129 scopus 로고    scopus 로고
    • Requirement of protein synthesis for group I mGluR-mediated induction of epileptiform discharges
    • Merlin LR, Bergold PJ, Wong RK: Requirement of protein synthesis for group I mGluR-mediated induction of epileptiform discharges. J Neurophysiol 80:989-993 (1998).
    • (1998) J Neurophysiol , vol.80 , pp. 989-993
    • Merlin, L.R.1    Bergold, P.J.2    Wong, R.K.3
  • 96
    • 0036197452 scopus 로고    scopus 로고
    • Behavioral and neuroanatomical characterization of the Fmr1 knockout mouse
    • Mineur YS, Sluyter F, de WS, Oostra BA, Crusio WE: Behavioral and neuroanatomical characterization of the Fmr1 knockout mouse. Hippocampus 12: 39-46 (2002).
    • (2002) Hippocampus , vol.12 , pp. 39-46
    • Mineur, Y.S.1    Sluyter, F.2    De, W.S.3    Oostra, B.A.4    Crusio, W.E.5
  • 97
    • 0025762624 scopus 로고
    • Pituitary-gonadal axis in prepubertal boys with the fragile X syndrome
    • Moore PS, Chudley AE, Winter JS: Pituitary-gonadal axis in prepubertal boys with the fragile X syndrome. Am J med Genet 39:374-375 (1991).
    • (1991) Am J Med Genet , vol.39 , pp. 374-375
    • Moore, P.S.1    Chudley, A.E.2    Winter, J.S.3
  • 99
    • 0025874384 scopus 로고
    • Proto-oncogene transcription factors and epilepsy
    • Morgan JI, Curran T: Proto-oncogene transcription factors and epilepsy. Trends Pharmacol Sci 12: 343-349 (1991).
    • (1991) Trends Pharmacol Sci , vol.12 , pp. 343-349
    • Morgan, J.I.1    Curran, T.2
  • 100
    • 0021173996 scopus 로고
    • Developments of a water-maze procedure for studying spatial learning in the rat
    • Morris R Developments of a water-maze procedure for studying spatial learning in the rat. J Neurosci Meth 11:47-60 (1984).
    • (1984) J Neurosci Meth , vol.11 , pp. 47-60
    • Morris, R.1
  • 101
    • 0019957779 scopus 로고
    • Place navigation impaired in rats with hippocampal lesions
    • Morris RG, Garrud P, Rawlins JN, O'Keefe J: Place navigation impaired in rats with hippocampal lesions. Nature 297:681-683 (1982).
    • (1982) Nature , vol.297 , pp. 681-683
    • Morris, R.G.1    Garrud, P.2    Rawlins, J.N.3    O'Keefe, J.4
  • 102
    • 0030837569 scopus 로고    scopus 로고
    • Glycine-induced long-term potentiation is associated with structural and functional modifications of alpha-amino-3-hydroxyl-5-methyl-4- isoxazolepropionic acid receptors
    • Musleh W, Bi X, Tocco G, Yaghoubi S, Baudry M: Glycine-induced long-term potentiation is associated with structural and functional modifications of alpha-amino-3-hydroxyl-5-methyl-4-isoxazolepropionic acid receptors. Proc natl Acad Sci, USA 94:9451-9456 (1997).
    • (1997) Proc Natl Acad Sci, USA , vol.94 , pp. 9451-9456
    • Musleh, W.1    Bi, X.2    Tocco, G.3    Yaghoubi, S.4    Baudry, M.5
  • 105
    • 0032514407 scopus 로고    scopus 로고
    • Maintenance of late-phase LTP is accompanied by PKA-dependent increase in AMPA receptor synthesis
    • Nayak A, Zastrow DJ, Lickteig R, Zahniser NR, Browning MD: Maintenance of late-phase LTP is accompanied by PKA-dependent increase in AMPA receptor synthesis. Nature 394:680-683 (1998).
    • (1998) Nature , vol.394 , pp. 680-683
    • Nayak, A.1    Zastrow, D.J.2    Lickteig, R.3    Zahniser, N.R.4    Browning, M.D.5
  • 106
    • 0037039527 scopus 로고    scopus 로고
    • Alterations in the auditory startle response in Fmr1 targeted mutant mouse models of fragile X syndrome
    • Nielsen DM, Derber WJ, McClellan DA, Crnic LS: Alterations in the auditory startle response in Fmr1 targeted mutant mouse models of fragile X syndrome. Brain Res 927:8-17 (2002).
    • (2002) Brain Res , vol.927 , pp. 8-17
    • Nielsen, D.M.1    Derber, W.J.2    McClellan, D.A.3    Crnic, L.S.4
  • 107
    • 0019968221 scopus 로고
    • Macroorchidism and fragile X in mentally retarded males. Clinical, cytogenetic, and some hormonal investigations in mentally retarded males, including two with the fragile site at Xq28, fra(X)(q28)
    • Nielsen KB, Tommerup N, Dyggve HV, Schou C: Macroorchidism and fragile X in mentally retarded males. Clinical, cytogenetic, and some hormonal investigations in mentally retarded males, including two with the fragile site at Xq28, fra(X)(q28). Hum Genet 61:113-117 (1982).
    • (1982) Hum Genet , vol.61 , pp. 113-117
    • Nielsen, K.B.1    Tommerup, N.2    Dyggve, H.V.3    Schou, C.4
  • 108
    • 0035879180 scopus 로고    scopus 로고
    • Abnormal development of dendritic spines in Fmr1 knock-out mice
    • Nimchinsky EA, Oberlander AM, Svoboda K: Abnormal development of dendritic spines in Fmr1 knock-out mice. J Neurosci 21:5139-5146 (2001).
    • (2001) J Neurosci , vol.21 , pp. 5139-5146
    • Nimchinsky, E.A.1    Oberlander, A.M.2    Svoboda, K.3
  • 110
    • 0019980538 scopus 로고
    • Proliferation of Sertoli cells in fetal and postnatal rats: A quantitative autoradiographic study
    • Orth JM: Proliferation of Sertoli cells in fetal and postnatal rats: a quantitative autoradiographic study. Anat Rec 203:485-492 (1982).
    • (1982) Anat Rec , vol.203 , pp. 485-492
    • Orth, J.M.1
  • 111
    • 0032881856 scopus 로고    scopus 로고
    • Fragile X mouse: Strain effects of knockout phenotype and evidence suggesting deficient amygdala function
    • Paradee W, Melikian HE, Rasmussen DL, Kenneson A, Conn PJ, Warren ST: Fragile X mouse: strain effects of knockout phenotype and evidence suggesting deficient amygdala function. Neuroscience 94:185-192 (1999).
    • (1999) Neuroscience , vol.94 , pp. 185-192
    • Paradee, W.1    Melikian, H.E.2    Rasmussen, D.L.3    Kenneson, A.4    Conn, P.J.5    Warren, S.T.6
  • 113
    • 0029853774 scopus 로고    scopus 로고
    • Tetraethylammonium-induced synaptic plasticity in rat neocortex
    • Pelletier MR, Hablitz JJ: Tetraethylammonium-induced synaptic plasticity in rat neocortex. Cereb Cortex 6:771-780 (1996).
    • (1996) Cereb Cortex , vol.6 , pp. 771-780
    • Pelletier, M.R.1    Hablitz, J.J.2
  • 115
    • 0034143238 scopus 로고    scopus 로고
    • Metabotropic glutamate receptors trigger homosynaptic protein synthesis to prolong long-term potentiation
    • Raymond CR, Thompson VL, Tate WP, Abraham WC: Metabotropic glutamate receptors trigger homosynaptic protein synthesis to prolong long-term potentiation. J Neurosci 20:969-976 (2000).
    • (2000) J Neurosci , vol.20 , pp. 969-976
    • Raymond, C.R.1    Thompson, V.L.2    Tate, W.P.3    Abraham, W.C.4
  • 118
    • 0026649793 scopus 로고
    • Behavioral phenotype of fragile X syndrome: DSM-III-R autistic behavior in male children
    • Reiss AL, Freund L: Behavioral phenotype of fragile X syndrome: DSM-III-R autistic behavior in male children. Am J med Genet 43:35-46 (1992).
    • (1992) Am J Med Genet , vol.43 , pp. 35-46
    • Reiss, A.L.1    Freund, L.2
  • 119
  • 120
    • 0023737463 scopus 로고
    • Preliminary communication: Neuronanatomical variations of the posterior fossa in men with the fragile X (Martin Bell) syndrome
    • Reiss AL, Patel S, Kumar AJ, Freund L: Preliminary communication: neuronanatomical variations of the posterior fossa in men with the fragile X (Martin Bell) syndrome. Am J med Genet 31:407-414 (1988).
    • (1988) Am J Med Genet , vol.31 , pp. 407-414
    • Reiss, A.L.1    Patel, S.2    Kumar, A.J.3    Freund, L.4
  • 123
    • 0026354010 scopus 로고
    • Selection in blood cells from female carriers of the fragile X syndrome: Inverse correlation between age and proportion of active X chromosomes carrying the full mutation
    • Rousseau F, Heitz D, Oberlé I, Mandel JL: Selection in blood cells from female carriers of the fragile X syndrome: inverse correlation between age and proportion of active X chromosomes carrying the full mutation. J med Genet 28:830-836 (1991b).
    • (1991) J Med Genet , vol.28 , pp. 830-836
    • Rousseau, F.1    Heitz, D.2    Oberlé, I.3    Mandel, J.L.4
  • 127
    • 0035801393 scopus 로고    scopus 로고
    • The fragile X mental retardation protein binds specifically to its mRNA via a purine quartet motif
    • Schaeffer C, Bardoni B, Mandel JL, Ehresmann B, Ehresmann C, Moine H: The fragile X mental retardation protein binds specifically to its mRNA via a purine quartet motif. Embo J 20:4803-4813 (2001).
    • (2001) Embo J , vol.20 , pp. 4803-4813
    • Schaeffer, C.1    Bardoni, B.2    Mandel, J.L.3    Ehresmann, B.4    Ehresmann, C.5    Moine, H.6
  • 128
    • 0028930184 scopus 로고
    • Correlations between radial-maze learning and structural variations of septum and hippocampus in rodents
    • Schwegler H, Crusio WE: Correlations between radial-maze learning and structural variations of septum and hippocampus in rodents. Behav Brain Res 67:29-41 (1995).
    • (1995) Behav Brain Res , vol.67 , pp. 29-41
    • Schwegler, H.1    Crusio, W.E.2
  • 129
    • 0025268240 scopus 로고
    • Hippocampal mossy fibers and radial-maze learning in the mouse: A correlation with spatial working memory but not with non-spatial reference memory
    • Schwegler H, Crusio WE, Brust I: Hippocampal mossy fibers and radial-maze learning in the mouse: a correlation with spatial working memory but not with non-spatial reference memory. Neuroscience 34: 293-298 (1990).
    • (1990) Neuroscience , vol.34 , pp. 293-298
    • Schwegler, H.1    Crusio, W.E.2    Brust, I.3
  • 130
    • 0001058431 scopus 로고
    • Experimental evidence for Sertoli-germ cell and Sertoli-Leydig cell interactions
    • Tussel LGD, Griswold MD (eds), Cache River Press, Clearwater
    • Sharpe RM: Experimental evidence for Sertoli-germ cell and Sertoli-Leydig cell interactions, in Russel LGD, Griswold MD (eds): The Sertoli cell, pp 391-418 (Cache River Press, Clearwater 1993).
    • (1993) The Sertoli Cell , pp. 391-418
    • Sharpe, R.M.1
  • 131
    • 0029983269 scopus 로고    scopus 로고
    • Cloning and characterization, and properties of plasmids containing CGG triplet repeats from the FMR-1 gene
    • Shimizu M, Gellibolian R, Oostra BA, Wells RD: Cloning and characterization, and properties of plasmids containing CGG triplet repeats from the FMR-1 gene. J molec Biol 258:614-626 (1996).
    • (1996) J Molec Biol , vol.258 , pp. 614-626
    • Shimizu, M.1    Gellibolian, R.2    Oostra, B.A.3    Wells, R.D.4
  • 132
    • 0026742451 scopus 로고
    • Impaired spatial learning in alpha-calcium-calmodulin kinase II mutant mice
    • Silva AJ, Paylor R, Wehner JM, Tonegawa S: Impaired spatial learning in alpha-calcium-calmodulin kinase II mutant mice. Science 257:206-211 (1992a).
    • (1992) Science , vol.257 , pp. 206-211
    • Silva, A.J.1    Paylor, R.2    Wehner, J.M.3    Tonegawa, S.4
  • 133
    • 0026637195 scopus 로고
    • Deficient hippocampal long-term potentiation in alpha-calcium-calmodulin kinase II mutant mice
    • Silva AJ, Stevens CF, Tonegawa S, Wang Y: Deficient hippocampal long-term potentiation in alpha-calcium-calmodulin kinase II mutant mice. Science 257:201-206 (1992b).
    • (1992) Science , vol.257 , pp. 201-206
    • Silva, A.J.1    Stevens, C.F.2    Tonegawa, S.3    Wang, Y.4
  • 134
    • 0028236525 scopus 로고
    • Essential role for KH domains in RNA binding: Impaired RNA binding by a mutation in the KH domain of FMR1 that causes fragile X syndrome
    • Siomi H, Choi M, Siomi MC, Nussbaum RL, Dreyfuss G: Essential role for KH domains in RNA binding: impaired RNA binding by a mutation in the KH domain of FMR1 that causes fragile X syndrome. Cell 77:33-39 (1994).
    • (1994) Cell , vol.77 , pp. 33-39
    • Siomi, H.1    Choi, M.2    Siomi, M.C.3    Nussbaum, R.L.4    Dreyfuss, G.5
  • 135
    • 0027327486 scopus 로고
    • The protein product of the fragile X gene, FMR1, has characteristics of an RNA-binding protein
    • Siomi H, Siomi MC, Nussbaum RL, Dreyfuss G: The protein product of the fragile X gene, FMR1, has characteristics of an RNA-binding protein. Cell 74:291-298 (1993).
    • (1993) Cell , vol.74 , pp. 291-298
    • Siomi, H.1    Siomi, M.C.2    Nussbaum, R.L.3    Dreyfuss, G.4
  • 136
    • 0030051618 scopus 로고    scopus 로고
    • Alternative splicing of exon 14 determines nuclear or cytoplasmic localization of FMR1 protein isoforms
    • Sittler A, Devys D, Weber C, Mandel J-L: Alternative splicing of exon 14 determines nuclear or cytoplasmic localization of FMR1 protein isoforms. Hum molec Genet 5:95-102 (1996).
    • (1996) Hum Molec Genet , vol.5 , pp. 95-102
    • Sittler, A.1    Devys, D.2    Weber, C.3    Mandel, J.-L.4
  • 139
    • 0028283366 scopus 로고
    • Prediction of mental status in carriers of the fragile X mutation using CGG repeat length
    • Smits A, Smeets D, Hamel B, Dreesen J, Dehaan A, Van Oost B: Prediction of mental status in carriers of the fragile X mutation using CGG repeat length. Am J med Genet 51:497-500 (1994).
    • (1994) Am J Med Genet , vol.51 , pp. 497-500
    • Smits, A.1    Smeets, D.2    Hamel, B.3    Dreesen, J.4    Dehaan, A.5    Van Oost, B.6
  • 141
    • 0032536361 scopus 로고    scopus 로고
    • No evidence for disruption of normal patterns of mRNA localization in dendrites or dendritic transport of recently synthesized mRNA in FMR1 knockout mice, a model for human fragile-X mental retardation syndrome
    • Steward O, Bakker CE, Willems PJ, Oostra BA: No evidence for disruption of normal patterns of mRNA localization in dendrites or dendritic transport of recently synthesized mRNA in FMR1 knockout mice, a model for human fragile-X mental retardation syndrome. Neuroreport 9:477-481 (1998).
    • (1998) Neuroreport , vol.9 , pp. 477-481
    • Steward, O.1    Bakker, C.E.2    Willems, P.J.3    Oostra, B.A.4
  • 145
    • 0033231022 scopus 로고    scopus 로고
    • Oligomerization properties of fragile-X mental-retardation protein (FMRP), and the fragile-X-related proteins FXR1P and FXR2P
    • Tamanini F, Van Unen L, Bakker C, Sacchi N, Galjaard H, Oostra BA, Hoogeveen AT: Oligomerization properties of fragile-X mental-retardation protein (FMRP). and the fragile-X-related proteins FXR1P and FXR2P. Biochem J 343:517-523 (1999).
    • (1999) Biochem J , vol.343 , pp. 517-523
    • Tamanini, F.1    Van Unen, L.2    Bakker, C.3    Sacchi, N.4    Galjaard, H.5    Oostra, B.A.6    Hoogeveen, A.T.7
  • 150
    • 0034106451 scopus 로고    scopus 로고
    • Linking Hebb's coincidence-detection to memory formation
    • Tsien JZ: Linking Hebb's coincidence-detection to memory formation. Curr Opin Neurobiol 10:266-273 (2000).
    • (2000) Curr Opin Neurobiol , vol.10 , pp. 266-273
    • Tsien, J.Z.1
  • 153
    • 0031115191 scopus 로고    scopus 로고
    • Model organisms illuminate human genetics and disease
    • van Heyningen V: Model organisms illuminate human genetics and disease. Mol Med 3:231-237 (1997).
    • (1997) Mol Med , vol.3 , pp. 231-237
    • Van Heyningen, V.1
  • 156
    • 0033761489 scopus 로고    scopus 로고
    • Characterization of dFMR1, a Drosophila melanogaster homolog of the Fragile X mental retardation protein
    • Wan L, Dockendorff TC, Jongens TA, Dreyfuss G: Characterization of dFMR1, a Drosophila melanogaster homolog of the Fragile X mental retardation protein. Mol Cell Biol 20:8536-8547 (2000).
    • (2000) Mol Cell Biol , vol.20 , pp. 8536-8547
    • Wan, L.1    Dockendorff, T.C.2    Jongens, T.A.3    Dreyfuss, G.4
  • 157
    • 0033515497 scopus 로고    scopus 로고
    • Synaptic synthesis of the Fragile X protein: Possible involvement in synapse maturation and elimination
    • Weiler IJ, Greenough WT: Synaptic synthesis of the Fragile X protein: possible involvement in synapse maturation and elimination. Am J med Genet 83:248-252 (1999).
    • (1999) Am J Med Genet , vol.83 , pp. 248-252
    • Weiler, I.J.1    Greenough, W.T.2
  • 159
    • 0030058075 scopus 로고    scopus 로고
    • Molecular basis of genetic instability of triplet repeats
    • Wells RD: Molecular basis of genetic instability of triplet repeats. J biol Chem 271:2875-2878 (1996).
    • (1996) J Biol Chem , vol.271 , pp. 2875-2878
    • Wells, R.D.1
  • 160
    • 0030836505 scopus 로고    scopus 로고
    • A survey of morphogenesis during the early postnatal period in PMBSF barrels of mouse SmI cortex with emphasis on barrel D4
    • White EL, Weinfeld L, Lev DL: A survey of morphogenesis during the early postnatal period in PMBSF barrels of mouse SmI cortex with emphasis on barrel D4. Somatosens Mot Res 14:34-55 (1997).
    • (1997) Somatosens Mot Res , vol.14 , pp. 34-55
    • White, E.L.1    Weinfeld, L.2    Lev, D.L.3
  • 161
    • 0032771002 scopus 로고    scopus 로고
    • n triplet repeat array in Saccharomyces cerevisiae deficient in aspects of DNA metabolism
    • n triplet repeat array in Saccharomyces cerevisiae deficient in aspects of DNA metabolism. Mol Cell Biol 19:5675-5684 (1999).
    • (1999) Mol Cell Biol , vol.19 , pp. 5675-5684
    • White, P.J.1    Borts, R.H.2    Hirst, M.C.3
  • 163
    • 0026781016 scopus 로고
    • A microdeletion of less than 250 kb, including the proximal part of the FMR-I gene and the fragile-X site, in a male with the clinical phenotype of fragile-X syndrome
    • Wöhrle D, Kotzot D, Hirst MC, Manca A, Korn B, Schmidt A, Barbi G, Rott HD, Poustka A, Davies KE, Steinbach P: A microdeletion of less than 250 kb, including the proximal part of the FMR-I gene and the fragile-X site, in a male with the clinical phenotype of fragile-X syndrome. Am J hum Genet 51:299-306 (1992).
    • (1992) Am J Hum Genet , vol.51 , pp. 299-306
    • Wöhrle, D.1    Kotzot, D.2    Hirst, M.C.3    Manca, A.4    Korn, B.5    Schmidt, A.6    Barbi, G.7    Rott, H.D.8    Poustka, A.9    Davies, K.E.10    Steinbach, P.11
  • 165
    • 0030576353 scopus 로고    scopus 로고
    • DNA methylation and triplet repeat stability: New proposals addressing actual questions on the CGG repeat of fragile X syndrome - Letter to the editor
    • Wohrle D, Schwemmle S, Steinbach P: DNA methylation and triplet repeat stability: New proposals addressing actual questions on the CGG repeat of
    • (1996) Am J Med Genet , vol.64 , pp. 266-267
    • Wohrle, D.1    Schwemmle, S.2    Steinbach, P.3
  • 166
    • 0032620105 scopus 로고    scopus 로고
    • Role of metabotropic glutamate receptors in epilepsy
    • Wong RK, Bianchi R, Taylor GW, Merlin LR: Role of metabotropic glutamate receptors in epilepsy. Adv Neurol 79:685-698 (1999).
    • (1999) Adv Neurol , vol.79 , pp. 685-698
    • Wong, R.K.1    Bianchi, R.2    Taylor, G.W.3    Merlin, L.R.4
  • 169


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