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Volumn 54, Issue , 2012, Pages 255-269

Mouse models of the fragile X premutation and the fragile X associated tremor/ataxia syndrome

Author keywords

[No Author keywords available]

Indexed keywords

FRAGILE X MENTAL RETARDATION PROTEIN;

EID: 80455174205     PISSN: 00801844     EISSN: 18610412     Source Type: Book Series    
DOI: 10.1007/978-3-642-21649-7_14     Document Type: Article
Times cited : (16)

References (72)
  • 1
    • 48249117487 scopus 로고    scopus 로고
    • Motor cortex stroke impairs individual digit movement in skilled reaching by the rat
    • Alaverdashvili M, Whishaw IQ (2008) Motor cortex stroke impairs individual digit movement in skilled reaching by the rat. Eur J Neurosci 28:311-322
    • (2008) Eur. J. Neurosci. , vol.28 , pp. 311-322
    • Alaverdashvili, M.1    Whishaw, I.Q.2
  • 3
    • 68549133201 scopus 로고    scopus 로고
    • Stepping test in mice: A reliable approach in determining forelimb akinesia in MPTP-induced Parkinsonism
    • Blume SR, Cass DK, Tseng KY (2009) Stepping test in mice: a reliable approach in determining forelimb akinesia in MPTP-induced Parkinsonism. Exp Neurol 219:208-211
    • (2009) Exp. Neurol. , vol.219 , pp. 208-211
    • Blume, S.R.1    Cass, D.K.2    Tseng, K.Y.3
  • 9
    • 56749165180 scopus 로고    scopus 로고
    • CGG-repeat length and neuropathological and molecular correlates in a mouse model for fragile X-associated tremor/ataxia syndrome
    • Brouwer JR, Huizer K, Severijnen LA, Hukema RK, Berman RF, Oostra BA, Willemsen R (2008a) CGG-repeat length and neuropathological and molecular correlates in a mouse model for fragile X-associated tremor/ataxia syndrome. J Neurochem 107:1671-1682
    • (2008) J. Neurochem. , vol.107 , pp. 1671-1682
    • Brouwer, J.R.1    Huizer, K.2    Severijnen, L.A.3    Hukema, R.K.4    Berman, R.F.5    Oostra, B.A.6    Willemsen, R.7
  • 10
    • 46249101217 scopus 로고    scopus 로고
    • Altered hypothalamus-pituitary-adrenal gland axis regulation in the expanded CGG-repeat mouse model for fragile X-associated tremor/ataxia syndrome
    • Brouwer JR, Severijnen E, de Jong FH, Hessl D, Hagerman RJ, Oostra BA, Willemsen R (2008b) Altered hypothalamus-pituitary-adrenal gland axis regulation in the expanded CGG-repeat mouse model for fragile X-associated tremor/ataxia syndrome. Psychoneuroendocrinology 33:863-873
    • (2008) Psychoneuroendocrinology , vol.33 , pp. 863-873
    • Brouwer, J.R.1    Severijnen, E.2    De Jong, F.H.3    Hessl, D.4    Hagerman, R.J.5    Oostra, B.A.6    Willemsen, R.7
  • 11
    • 56749165180 scopus 로고    scopus 로고
    • CGG repeat length and neuropathological and molecular correlates in a mouse model for fragile Xassociated tremor/ataxia syndrome
    • Brouwer JR, Huizer K, Severijnen LA, Hukema RK, Berman RF, Oostra WR (2008c) CGG repeat length and neuropathological and molecular correlates in a mouse model for fragile Xassociated tremor/ataxia syndrome. J Neurochem 107:1671-1682
    • (2008) J. Neurochem. , vol.107 , pp. 1671-1682
    • Brouwer, J.R.1    Huizer, K.2    Severijnen, L.A.3    Hukema, R.K.4    Berman, R.F.5    Oostra, W.R.6
  • 14
    • 77649301567 scopus 로고    scopus 로고
    • Murine hippocampal neurons expressing Fmr1 gene premutations show early developmental deficits and late degeneration
    • Chen Y, Tassone F, Berman RF, Hagerman P, Hagerman RJ, Willemsen R, Pessah IN (2010) Murine hippocampal neurons expressing Fmr1 gene premutations show early developmental deficits and late degeneration. Hum Mol Genet 19:196-208
    • (2010) Hum. Mol. Genet. , vol.19 , pp. 196-208
    • Chen, Y.1    Tassone, F.2    Berman, R.F.3    Hagerman, P.4    Hagerman, R.J.5    Willemsen, R.6    Pessah, I.N.7
  • 15
    • 11144293459 scopus 로고    scopus 로고
    • The emerging fragile X premutation phenotype: Evidence from the domain of social cognition
    • Cornish K, Kogan C, Turk J, Manly T, James N, Mills A, Dalton A (2005) The emerging fragile X premutation phenotype: evidence from the domain of social cognition. Brain Cogn 57:53-60
    • (2005) Brain Cogn. , vol.57 , pp. 53-60
    • Cornish, K.1    Kogan, C.2    Turk, J.3    Manly, T.4    James, N.5    Mills, A.6    Dalton, A.7
  • 21
    • 39549086548 scopus 로고    scopus 로고
    • ATR protects the genome against CGG.CCG-repeat expansion in Fragile X premutation mice
    • Entezam A, Usdin K (2008) ATR protects the genome against CGG.CCG-repeat expansion in Fragile X premutation mice. Nucleic Acids Res 36:1050-1056
    • (2008) Nucleic Acids Res. , vol.36 , pp. 1050-1056
    • Entezam, A.1    Usdin, K.2
  • 22
    • 71049195737 scopus 로고    scopus 로고
    • ATM and ATR protect the genome against two different types of tandem repeat instability in Fragile X premutation mice
    • Entezam A, Usdin K (2009) ATM and ATR protect the genome against two different types of tandem repeat instability in Fragile X premutation mice. Nucleic Acids Res 37:6371-6377
    • (2009) Nucleic Acids Res. , vol.37 , pp. 6371-6377
    • Entezam, A.1    Usdin, K.2
  • 23
    • 34247637636 scopus 로고    scopus 로고
    • Regional FMRP deficits and large repeat expansions into the full mutation range in a new fragile X premutation mouse model
    • Entezam A, Biacsi R, Orrison B, Saha T, Hoffman GE, Grabczyk E, Nussbaum RL, Usdin K (2007) Regional FMRP deficits and large repeat expansions into the full mutation range in a new fragile X premutation mouse model. Gene 395:125-134
    • (2007) Gene , vol.395 , pp. 125-134
    • Entezam, A.1    Biacsi, R.2    Orrison, B.3    Saha, T.4    Hoffman, G.E.5    Grabczyk, E.6    Nussbaum, R.L.7    Usdin, K.8
  • 24
    • 77951773059 scopus 로고    scopus 로고
    • Potassium bromate a potent DNA oxidizing agent exacerbates germline repeat expansion in a fragile X premutation mouse model
    • Entezam A, Lokanga AR, Le W, Hoffman G, Usdin K (2010) Potassium bromate, a potent DNA oxidizing agent, exacerbates germline repeat expansion in a fragile X premutation mouse model. Hum Mutat 31:611-616
    • (2010) Hum. Mutat. , vol.31 , pp. 611-616
    • Entezam, A.1    Lokanga, A.R.2    Le W Hoffman, G.3    Usdin, K.4
  • 25
    • 0036310689 scopus 로고    scopus 로고
    • Quantitative and qualitative impairments in skilled reaching in the mouse Mus musculus after a focal motor cortex stroke
    • Farr TD, Whishaw IQ (2002) Quantitative and qualitative impairments in skilled reaching in the mouse (Mus musculus) after a focal motor cortex stroke. Stroke 33:1869-1875
    • (2002) Stroke , vol.33 , pp. 1869-1875
    • Farr, T.D.1    Whishaw, I.Q.2
  • 26
    • 33646145517 scopus 로고    scopus 로고
    • Bilateral alteration in stepping pattern after unilateral motor cortex injury: A new test strategy for analysis of skilled limb movements in neurological mouse models
    • Farr TD, Liu L, Colwell KL, Whishaw IQ, Metz GA (2006) Bilateral alteration in stepping pattern after unilateral motor cortex injury: a new test strategy for analysis of skilled limb movements in neurological mouse models. J Neurosci Methods 153:104-113
    • (2006) J. Neurosci. Methods , vol.153 , pp. 104-113
    • Farr, T.D.1    Liu, L.2    Colwell, K.L.3    Whishaw, I.Q.4    Metz, G.A.5
  • 27
    • 77953884301 scopus 로고    scopus 로고
    • Advances in understanding the molecular basis of FXTAS
    • Garcia A, Hagerman P (2010) Advances in understanding the molecular basis of FXTAS. Hum Mol Genet 19:R83-R89
    • (2010) Hum. Mol. Genet. , vol.19
    • Garcia, A.1    Hagerman, P.2
  • 28
    • 77953884301 scopus 로고    scopus 로고
    • Advances in understanding the molecular basis of FXTAS
    • Garcia-Arocena D, Hagerman PJ (2010) Advances in understanding the molecular basis of FXTAS. Hum Mol Genet 19:R83-R89
    • (2010) Hum. Mol. Genet. , vol.19
    • Garcia-Arocena, D.1    Hagerman, P.J.2
  • 29
    • 77951183832 scopus 로고    scopus 로고
    • Lesions to the ventral but not the dorsal medial prefrontal cortex enhance latent inhibition
    • George DN, Duffaud AM, Pothuizen HH, Haddon JE, Killcross S (2010) Lesions to the ventral, but not the dorsal, medial prefrontal cortex enhance latent inhibition. Eur J Neurosci 31: 1474-1482
    • (2010) Eur. J. Neurosci. , vol.31 , pp. 1474-1482
    • George, D.N.1    Duffaud, A.M.2    Pothuizen, H.H.3    Haddon, J.E.4    Killcross, S.5
  • 30
    • 27744521339 scopus 로고    scopus 로고
    • Dissociating the role of the parietal cortex and dorsal hippocampus for spatial information processing
    • Goodrich-Hunsaker NJ, Hunsaker MR, Kesner RP (2005) Dissociating the role of the parietal cortex and dorsal hippocampus for spatial information processing. Behav Neurosci 119: 1307-1315
    • (2005) Behav. Neurosci. , vol.119 , pp. 1307-1315
    • Goodrich-Hunsaker, N.J.1    Hunsaker, M.R.2    Kesner, R.P.3
  • 31
  • 36
    • 49449105825 scopus 로고    scopus 로고
    • Contextual control of biconditional task performance: Evidence for cue and response competition in rats
    • Haddon JE, George DN, Killcross S (2008) Contextual control of biconditional task performance: evidence for cue and response competition in rats. Q J Exp Psychol (Colchester) 61:1307-1320
    • (2008) Q. J. Exp. Psychol. Colchester , vol.61 , pp. 1307-1320
    • Haddon, J.E.1    George, D.N.2    Killcross, S.3
  • 37
    • 50049086691 scopus 로고    scopus 로고
    • The fragile X prevalence paradox
    • Hagerman PJ (2008) The fragile X prevalence paradox. J Med Genet 45:498-499
    • (2008) J. Med. Genet. , vol.45 , pp. 498-499
    • Hagerman, P.J.1
  • 38
    • 2342635196 scopus 로고    scopus 로고
    • The fragile-X premutation: A maturing perspective
    • Hagerman P, Hagerman RJ (2004) The fragile-X premutation: a maturing perspective. Am J Hum Genet 74:805-816
    • (2004) Am. J. Hum. Genet. , vol.74 , pp. 805-816
    • Hagerman, P.1    Hagerman, R.J.2
  • 41
    • 72249123025 scopus 로고    scopus 로고
    • Progressive spatial processing deficits in a mouse model of the fragile X premutation
    • Hunsaker MR, Wenzel HJ, Willemsen R, Berman RF (2009) Progressive spatial processing deficits in a mouse model of the fragile X premutation. Behav Neurosci 123:1315-1324
    • (2009) Behav. Neurosci. , vol.123 , pp. 1315-1324
    • Hunsaker, M.R.1    Wenzel, H.J.2    Willemsen, R.3    Berman, R.F.4
  • 42
    • 77953917097 scopus 로고    scopus 로고
    • Temporal ordering deficits in female CGG KI mice heterozygous for the fragile X premutation
    • Hunsaker MR, Goodrich-Hunsaker NJ, Willemsen R, Berman RF (2010) Temporal ordering deficits in female CGG KI mice heterozygous for the fragile X premutation. Behav Brain Res 213:263-268
    • (2010) Behav. Brain Res. , vol.213 , pp. 263-268
    • Hunsaker, M.R.1    Goodrich-Hunsaker, N.J.2    Willemsen, R.3    Berman, R.F.4
  • 45
    • 0035394437 scopus 로고    scopus 로고
    • Reduced FMRP and increased FMR1 transcription is proportionally associated with CGG repeat number in intermediatelength and premutation carriers
    • Kenneson A, Zhang F, Hagedorn CH, Warren ST (2001) Reduced FMRP and increased FMR1 transcription is proportionally associated with CGG repeat number in intermediatelength and premutation carriers. Hum Mol Genet 10:1449-1454
    • (2001) Hum. Mol. Genet. , vol.10 , pp. 1449-1454
    • Kenneson, A.1    Zhang, F.2    Hagedorn, C.H.3    Warren, S.T.4
  • 47
    • 0030931869 scopus 로고    scopus 로고
    • Trinucleotide repeats CGG 22TGG CGG 43TGG CGG 21 from the fragile X gene remain stable in transgenic mice
    • Lavedan CN, Garrett L, Nussbaum RL (1997) Trinucleotide repeats (CGG)22TGG(CGG)43TGG (CGG)21 from the fragile X gene remain stable in transgenic mice. Hum Genet 100:407-414
    • (1997) Hum. Genet. , vol.100 , pp. 407-414
    • Lavedan, C.N.1    Garrett, L.2    Nussbaum, R.L.3
  • 48
    • 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 (1998) 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) Genomics , vol.50 , pp. 229-240
    • Lavedan, C.1    Grabczyk, E.2    Usdin, K.3    Nussbaum, R.L.4
  • 51
    • 33847043593 scopus 로고    scopus 로고
    • Inactivation of the prelimbic but not infralimbic prefrontal cortex impairs the contextual control of response conflict in rats
    • Marquis JP, Killcross S, Haddon JE (2007) Inactivation of the prelimbic, but not infralimbic, prefrontal cortex impairs the contextual control of response conflict in rats. Eur J Neurosci 25:559-566
    • (2007) Eur. J. Neurosci. , vol.25 , pp. 559-566
    • Marquis, J.P.1    Killcross, S.2    Haddon, J.E.3
  • 52
    • 0037091202 scopus 로고    scopus 로고
    • Cortical and subcortical lesions impair skilled walking in the ladder rung walking test: A new task to evaluate fore-and hindlimb stepping placing and coordination
    • Metz GA, Whishaw IQ (2002) Cortical and subcortical lesions impair skilled walking in the ladder rung walking test: a new task to evaluate fore- and hindlimb stepping, placing, and coordination. J Neurosci Methods 115:169-179
    • (2002) J. Neurosci. Methods , vol.115 , pp. 169-179
    • Metz, G.A.1    Whishaw, I.Q.2
  • 53
    • 80055119762 scopus 로고    scopus 로고
    • The ladder rung walking task: A scoring system and its practical application
    • Metz GA, Whishaw IQ (2009) The ladder rung walking task: a scoring system and its practical application. J Vis Exp 12:1204
    • (2009) J. Vis. Exp. , vol.12 , pp. 1204
    • Metz, G.A.1    Whishaw, I.Q.2
  • 55
    • 79751535827 scopus 로고    scopus 로고
    • A mouse model of the fragile X premutation: Effects on behavior dendrite morphology and regional rates of cerebral protein synthesis
    • Qin M, Entezam A, Usdin K, Huang T, Liu ZH, Hoffman GE, Smith CB (2011) A mouse model of the fragile X premutation: effects on behavior, dendrite morphology, and regional rates of cerebral protein synthesis. Neurobiol Dis 42:85-98
    • (2011) Neurobiol. Dis. , vol.42 , pp. 85-98
    • Qin, M.1    Entezam, A.2    Usdin, K.3    Huang, T.4    Liu, Z.H.5    Hoffman, G.E.6    Smith, C.B.7
  • 56
    • 74249089729 scopus 로고    scopus 로고
    • Molecular pathogenesis of fragile X-Associated tremor/ataxia syndrome
    • Raske C, Hagerman PJ (2009) Molecular pathogenesis of fragile X-Associated tremor/ataxia syndrome. J Investig Med 57(8):825-829
    • (2009) J. Investig. Med. , vol.57 , Issue.8 , pp. 825-829
    • Raske, C.1    Hagerman, P.J.2
  • 58
    • 67649095407 scopus 로고    scopus 로고
    • Covariate adjusted correlation analysis with application to FMR1 premutation female carrier data
    • Senturk D, Nguyen DV, Tassone F, Hagerman RJ, Carroll RJ, Hagerman PJ (2009) Covariate adjusted correlation analysis with application to FMR1 premutation female carrier data. Biometrics 65:781-792
    • (2009) Biometrics , vol.65 , pp. 781-792
    • Senturk, D.1    Nguyen, D.V.2    Tassone, F.3    Hagerman, R.J.4    Carroll, R.J.5    Hagerman, P.J.6
  • 60
    • 0034684031 scopus 로고    scopus 로고
    • Fragile X males with unmethylated full mutation trinucleotide repeat expansions have elevated levels of FMR1 messenger RNA
    • Tassone F, Hagerman RJ, Loesch DZ, Lachiewicz A, Taylor AK, Hagerman PJ (2000a) Fragile X males with unmethylated, full mutation trinucleotide repeat expansions have elevated levels of FMR1 messenger RNA. Am J Med Genet 94:232-236
    • (2000) Am. J. Med. Genet. , vol.94 , pp. 232-236
    • Tassone, F.1    Hagerman, R.J.2    Loesch, D.Z.3    Lachiewicz, A.4    Taylor, A.K.5    Hagerman, P.J.6
  • 64
    • 23944431645 scopus 로고    scopus 로고
    • FMR1 RNA within the intranuclear inclusions of fragile X-associated tremor/ataxia syndrome FXTAS
    • Tassone F, Iwahashi C, Hagerman PJ (2004b) FMR1 RNA within the intranuclear inclusions of fragile X-associated tremor/ataxia syndrome (FXTAS). RNA Biol 1:103-105
    • (2004) RNA Biol. , vol.1 , pp. 103-105
    • Tassone, F.1    Iwahashi, C.2    Hagerman, P.J.3
  • 66
    • 20444447397 scopus 로고    scopus 로고
    • Cognitive decline neuromotor and behavioural disturbances in a mouse model for fragile-Xassociated tremor/ataxia syndrome FXTAS
    • Van Dam D, Errijgers V, Kooy RF, Willemsen R, Mientjes E, Oostra BA, De Deyn PP (2005) Cognitive decline, neuromotor and behavioural disturbances in a mouse model for fragile-Xassociated tremor/ataxia syndrome (FXTAS). Behav Brain Res 162:233-239
    • (2005) Behav. Brain Res. , vol.162 , pp. 233-239
    • Van Dam, D.1    Errijgers, V.2    Kooy, R.F.3    Willemsen, R.4    Mientjes, E.5    Oostra, B.A.6    De Deyn, P.P.7
  • 67
    • 0031475149 scopus 로고    scopus 로고
    • Deficits in response initiation but not attention following excitotoxic lesions of posterior parietal cortex in the rat
    • Ward B (1997) Deficits in response initiation, but not attention, following excitotoxic lesions of posterior parietal cortex in the rat. Brain Res 775:81-90
    • (1997) Brain Res. , vol.775 , pp. 81-90
    • Ward, B.1
  • 68
    • 0029985962 scopus 로고    scopus 로고
    • Covert orienting of attention in the rat and the role of striatal dopamine
    • Ward NM, Brown VJ (1996) Covert orienting of attention in the rat and the role of striatal dopamine. J Neurosci 16:3082-3088
    • (1996) J. Neurosci. , vol.16 , pp. 3082-3088
    • Ward, N.M.1    Brown, V.J.2
  • 69
    • 76449105593 scopus 로고    scopus 로고
    • Ubiquitinpositive intranuclear inclusions in neuronal and glial cells in a mouse model of the fragile X premutation
    • Wenzel HJ, Hunsaker MR, Greco CM, Willemsen R, Berman RF (2010) Ubiquitinpositive intranuclear inclusions in neuronal and glial cells in a mouse model of the fragile X premutation. Brain Res 1318:155-166
    • (2010) Brain Res. , vol.1318 , pp. 155-166
    • Wenzel, H.J.1    Hunsaker, M.R.2    Greco, C.M.3    Willemsen, R.4    Berman, R.F.5
  • 70
    • 0037151286 scopus 로고    scopus 로고
    • Absence of impairments or recovery mediated by the uncrossed pyramidal tract in the rat versus enduring deficits produced by the crossed pyramidal tract
    • Whishaw IQ, Metz GA (2002) Absence of impairments or recovery mediated by the uncrossed pyramidal tract in the rat versus enduring deficits produced by the crossed pyramidal tract. Behav Brain Res 134:323-336
    • (2002) Behav. Brain Res. , vol.134 , pp. 323-336
    • Whishaw, I.Q.1    Metz, G.A.2
  • 71
    • 70350211449 scopus 로고    scopus 로고
    • Hand shaping in the rat: Conserved release and collection vs. flexible manipulation in overground walking, ladder rung walking cylinder exploration and skilled reaching
    • Whishaw IQ, Travis SG, Koppe SW, Sacrey LA, Gholamrezaei G, Gorny B (2010) Hand shaping in the rat: conserved release and collection vs. flexible manipulation in overground walking, ladder rung walking, cylinder exploration, and skilled reaching. Behav Brain Res 206:21-31
    • (2010) Behav. Brain Res. , vol.206 , pp. 21-31
    • Whishaw, I.Q.1    Travis, S.G.2    Koppe, S.W.3    Sacrey, L.A.4    Gholamrezaei, G.5    Gorny, B.6


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.