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Volumn 4, Issue 7, 2015, Pages 800-808

High-throughput screening to identify compounds that increase fragile x mental retardation protein expression in neural stem cells differentiated from fragile x syndrome patient-derived induced pluripotent stem cells

Author keywords

Fibroblasts; Fragile X mental retardation protein assay; Fragile X syndrome; High throughput screening; Induced pluripotent stem cells; Neural stem cells

Indexed keywords

FRAGILE X MENTAL RETARDATION PROTEIN;

EID: 84936996242     PISSN: 21576564     EISSN: 21576580     Source Type: Journal    
DOI: 10.5966/sctm.2014-0278     Document Type: Article
Times cited : (71)

References (42)
  • 2
    • 77954915376 scopus 로고    scopus 로고
    • Fragile X: Leading the way for targeted treatments in autism
    • Wang LW, Berry-Kravis E, Hagerman RJ. Fragile X: Leading the way for targeted treatments in autism. Neurotherapeutics 2010; 7: 264-274.
    • (2010) Neurotherapeutics , vol.7 , pp. 264-274
    • Wang, L.W.1    Berry-Kravis, E.2    Hagerman, R.J.3
  • 3
    • 0026345716 scopus 로고
    • Variation of the CGG repeat at the fragile X site results in genetic instability: Resolution of the Sherman paradox
    • Fu YH, Kuhl DP, Pizzuti A et al. Variation of the CGG repeat at the fragile X site results in genetic instability: Resolution of the Sherman paradox. Cell 1991; 67: 1047-1058.
    • (1991) Cell , vol.67 , pp. 1047-1058
    • Fu, Y.H.1    Kuhl, D.P.2    Pizzuti, A.3
  • 4
    • 0025905795 scopus 로고
    • Identification of a gene (FMR-1) containing aCGGrepeatcoincidentwithabreakpoint cluster region exhibiting length variation in fragile X syndrome
    • Verkerk AJ, Pieretti M, Sutcliffe JS et al. Identification of a gene (FMR-1) containing aCGGrepeatcoincidentwithabreakpoint cluster region exhibiting length variation in fragile X syndrome. Cell 1991; 65: 905-914.
    • (1991) Cell , vol.65 , pp. 905-914
    • Verkerk, A.J.1    Pieretti, M.2    Sutcliffe, J.S.3
  • 5
    • 0036782129 scopus 로고    scopus 로고
    • Histone modifications depict an aberrantly heterochromatinized FMR1 gene in fragile X syndrome
    • Coffee B, Zhang F, Ceman S et al. Histone modifications depict an aberrantly heterochromatinized FMR1 gene in fragile X syndrome. Am J Hum Genet 2002; 71: 923-932.
    • (2002) Am J Hum Genet , vol.71 , pp. 923-932
    • Coffee, B.1    Zhang, F.2    Ceman, S.3
  • 6
    • 0026922707 scopus 로고
    • DNA methylation represses FMR-1 transcription in fragile X syndrome
    • Sutcliffe JS, Nelson DL, Zhang F et al. DNA methylation represses FMR-1 transcription in fragile X syndrome. Hum Mol Genet 1992; 1: 397-400.
    • (1992) Hum Mol Genet , vol.1 , pp. 397-400
    • Sutcliffe, J.S.1    Nelson, D.L.2    Zhang, F.3
  • 7
    • 84871413198 scopus 로고    scopus 로고
    • FMRPtargets distinctmRNAsequence elements to regulate protein expression
    • Ascano M Jr., Mukherjee N, Bandaru P et al. FMRPtargets distinctmRNAsequence elements to regulate protein expression. Nature 2012; 492: 382-386.
    • (2012) Nature , vol.492 , pp. 382-386
    • Ascano, M.1    Mukherjee, N.2    Bandaru, P.3
  • 8
    • 18044379515 scopus 로고    scopus 로고
    • Microarray identification of FMRP-associated brain mRNAs and altered mRNA translational profiles in fragile X syndrome
    • Brown V, Jin P, Ceman S et al. Microarray identification of FMRP-associated brain mRNAs and altered mRNA translational profiles in fragile X syndrome. Cell 2001; 107: 477-487.
    • (2001) Cell , vol.107 , pp. 477-487
    • Brown, V.1    Jin, P.2    Ceman, S.3
  • 9
    • 79960779323 scopus 로고    scopus 로고
    • FMRP stalls ribosomal translocation on mRNAs linked to synaptic function and autism
    • Darnell JC, Van Driesche SJ, Zhang C et al. FMRP stalls ribosomal translocation on mRNAs linked to synaptic function and autism. Cell 2011; 146: 247-261.
    • (2011) Cell , vol.146 , pp. 247-261
    • Darnell, J.C.1    Van Driesche, S.J.2    Zhang, C.3
  • 10
    • 0029816723 scopus 로고    scopus 로고
    • The fragile X mental retardation protein is a ribonucleoprotein containing both nuclear localization and nuclear export signals
    • Eberhart DE, Malter HE, Feng Y et al. The fragile X mental retardation protein is a ribonucleoprotein containing both nuclear localization and nuclear export signals. Hum Mol Genet 1996; 5: 1083-1091.
    • (1996) Hum Mol Genet , vol.5 , pp. 1083-1091
    • Eberhart, D.E.1    Malter, H.E.2    Feng, Y.3
  • 11
    • 77953249227 scopus 로고    scopus 로고
    • hnRNP C promotes APP translation by competing with FMRP for APP mRNA recruitment to P bodies
    • Lee EK, Kim HH, Kuwano Y et al. hnRNP C promotes APP translation by competing with FMRP for APP mRNA recruitment to P bodies. Nat Struct Mol Biol 2010; 17: 732-739.
    • (2010) Nat Struct Mol Biol , vol.17 , pp. 732-739
    • Lee, E.K.1    Kim, H.H.2    Kuwano, Y.3
  • 12
    • 79751527394 scopus 로고    scopus 로고
    • Cellular stressinduced up-regulation of FMRP promotes cell survival bymodulating PI3K-Akt phosphorylation cascades
    • JeonSJ, Seo JE, Yang SI etal. Cellular stressinduced up-regulation of FMRP promotes cell survival bymodulating PI3K-Akt phosphorylation cascades. J Biomed Sci 2011; 18: 17.
    • (2011) J Biomed Sci , vol.18 , pp. 17
    • Jeon, S.J.1    Seo, J.E.2    Yang, S.I.3
  • 13
    • 84884926024 scopus 로고    scopus 로고
    • The fragile X protein binds mRNAs involved in cancer progression and modulates metastasis formation
    • Luća R, Averna M, Zalfa F et al. The fragile X protein binds mRNAs involved in cancer progression and modulates metastasis formation. EMBO Mol Med 2013; 5: 1523-1536.
    • (2013) EMBO Mol Med , vol.5 , pp. 1523-1536
    • Luća, R.1    Averna, M.2    Zalfa, F.3
  • 14
    • 84900333592 scopus 로고    scopus 로고
    • Achromatin-dependent role of the fragile X mental retardation protein FMRP in the DNAdamage response
    • Alpatov R, Lesch BJ, Nakamoto-Kinoshita Met al. Achromatin-dependent role of the fragile X mental retardation protein FMRP in the DNAdamage response. Cell 2014; 157: 869-881.
    • (2014) Cell , vol.157 , pp. 869-881
    • Alpatov, R.1    Lesch, B.J.2    Nakamoto-Kinoshita, M.3
  • 15
    • 84907838387 scopus 로고    scopus 로고
    • A feedforward mechanism involving Drosophila fragile X mental retardation protein triggers a replication stress-induced DNA damage response
    • Zhang W, Cheng Y, Li Y et al. A feedforward mechanism involving Drosophila fragile X mental retardation protein triggers a replication stress-induced DNA damage response. Hum Mol Genet 2014; 23: 5188-5196.
    • (2014) Hum Mol Genet , vol.23 , pp. 5188-5196
    • Zhang, W.1    Cheng, Y.2    Li, Y.3
  • 16
    • 0031046778 scopus 로고    scopus 로고
    • Fragile X mental retardation protein: Nucleocytoplasmic shuttling and association with somatodendritic ribosomes
    • Feng Y, Gutekunst CA, Eberhart DE et al. Fragile X mental retardation protein: Nucleocytoplasmic shuttling and association with somatodendritic ribosomes. J Neurosci 1997; 17: 1539-1547.
    • (1997) J Neurosci , vol.17 , pp. 1539-1547
    • Feng, Y.1    Gutekunst, C.A.2    Eberhart, D.E.3
  • 17
    • 84886953546 scopus 로고    scopus 로고
    • The translation of translational control by FMRP: Therapeutic targets for FXS
    • Darnell JC, Klann E. The translation of translational control by FMRP: Therapeutic targets for FXS. Nat Neurosci 2013; 16: 1530-1536.
    • (2013) Nat Neurosci , vol.16 , pp. 1530-1536
    • Darnell, J.C.1    Klann, E.2
  • 18
    • 0028264043 scopus 로고
    • High functioning fragile X males: Demonstration of an unmethylated fully expanded FMR-1. mutation associated with protein expression
    • Hagerman RJ, Hull CE, Safanda JF et al. High functioning fragile X males: Demonstration of an unmethylated fully expanded FMR-1. mutation associated with protein expression. Am J Med Genet 1994; 51: 298-308.
    • (1994) Am J Med Genet , vol.51 , pp. 298-308
    • Hagerman, R.J.1    Hull, C.E.2    Safanda, J.F.3
  • 19
    • 79955682738 scopus 로고    scopus 로고
    • Ablation of FMRP in adult neural stem cells disrupts hippocampus-dependent learning
    • Guo W, Allan AM, Zong R et al. Ablation of FMRP in adult neural stem cells disrupts hippocampus-dependent learning. Nat Med 2011; 17: 559-565.
    • (2011) Nat Med , vol.17 , pp. 559-565
    • Guo, W.1    Allan, A.M.2    Zong, R.3
  • 20
    • 0031985868 scopus 로고    scopus 로고
    • In vitro reactivation of the FMR1 gene involved in fragile X syndrome
    • Chiurazzi P, Pomponi MG, Willemsen R et al. In vitro reactivation of the FMR1 gene involved in fragile X syndrome. Hum Mol Genet 1998; 7: 109-113.
    • (1998) Hum Mol Genet , vol.7 , pp. 109-113
    • Chiurazzi, P.1    Pomponi, M.G.2    Willemsen, R.3
  • 21
    • 41949125454 scopus 로고    scopus 로고
    • SIRT1 inhibition alleviates gene silencing in fragile X mental retardation syndrome
    • Biacsi R, Kumari D, Usdin K. SIRT1 inhibition alleviates gene silencing in fragile X mental retardation syndrome. PLoS Genet 2008; 4: e1000017.
    • (2008) PLoS Genet , vol.4
    • Biacsi, R.1    Kumari, D.2    Usdin, K.3
  • 22
    • 84879068188 scopus 로고    scopus 로고
    • Fragile X-associated tremor/ataxia syndrome (FXTAS): Pathology and mechanisms
    • Hagerman P. Fragile X-associated tremor/ataxia syndrome (FXTAS): Pathology and mechanisms. Acta Neuropathol 2013; 126: 1-19.
    • (2013) Acta Neuropathol , vol.126 , pp. 1-19
    • Hagerman, P.1
  • 23
    • 34547813683 scopus 로고    scopus 로고
    • Examination of reproductive aging milestones among women who carry the FMR1 premutation
    • Allen EG, Sullivan AK, Marcus M et al. Examination of reproductive aging milestones among women who carry the FMR1 premutation. Hum Reprod 2007; 22: 2142-2152.
    • (2007) Hum Reprod , vol.22 , pp. 2142-2152
    • Allen, E.G.1    Sullivan, A.K.2    Marcus, M.3
  • 24
    • 0034522229 scopus 로고    scopus 로고
    • Premature ovarian failure in the fragile X syndrome
    • Sherman SL. Premature ovarian failure in the fragile X syndrome. Am J Med Genet 2000; 97: 189-194.
    • (2000) Am J Med Genet , vol.97 , pp. 189-194
    • Sherman, S.L.1
  • 26
    • 34547681603 scopus 로고    scopus 로고
    • Pur alpha binds to rCGG repeats and modulates repeatmediated neurodegeneration in a Drosophila model of fragile X tremor/ataxia syndrome
    • Jin P, Duan R, Qurashi A et al. Pur alpha binds to rCGG repeats and modulates repeatmediated neurodegeneration in a Drosophila model of fragile X tremor/ataxia syndrome. Neuron 2007; 55: 556-564.
    • (2007) Neuron , vol.55 , pp. 556-564
    • Jin, P.1    Duan, R.2    Qurashi, A.3
  • 27
    • 84875804170 scopus 로고    scopus 로고
    • Sequestration of DROSHA and DGCR8 by expanded CGG RNA repeats alters microRNA processing in fragile X-associated tremor/ataxia syndrome
    • Sellier C, Freyermuth F, Tabet R et al. Sequestration of DROSHA and DGCR8 by expanded CGG RNA repeats alters microRNA processing in fragile X-associated tremor/ataxia syndrome. Cell Reports 2013; 3: 869-880.
    • (2013) Cell Reports , vol.3 , pp. 869-880
    • Sellier, C.1    Freyermuth, F.2    Tabet, R.3
  • 28
    • 77950529507 scopus 로고    scopus 로고
    • Sam68 sequestration and partial loss of function are associated with splicing alterations in FXTAS patients
    • Sellier C, Rau F, Liu Y et al. Sam68 sequestration and partial loss of function are associated with splicing alterations in FXTAS patients. EMBO J 2010; 29: 1248-1261.
    • (2010) EMBO J , vol.29 , pp. 1248-1261
    • Sellier, C.1    Rau, F.2    Liu, Y.3
  • 29
    • 84877331220 scopus 로고    scopus 로고
    • CGGrepeatassociated translation mediates neurodegeneration in fragile X tremor ataxia syndrome
    • Todd PK, OhSY, KransAet al. CGGrepeatassociated translation mediates neurodegeneration in fragile X tremor ataxia syndrome. Neuron 2013; 78: 440-455.
    • (2013) Neuron , vol.78 , pp. 440-455
    • Todd, P.K.1    Oh, S.Y.2    Krans, A.3
  • 30
    • 0035394437 scopus 로고    scopus 로고
    • Reduced FMRP and increased FMR1 transcription is proportionally associated with CGG repeat number in intermediate-length and premutation carriers
    • Kenneson A, Zhang F, Hagedorn CH et al. Reduced FMRP and increased FMR1 transcription is proportionally associated with CGG repeat number in intermediate-length and premutation carriers. Hum Mol Genet 2001; 10: 1449-1454.
    • (2001) Hum Mol Genet , vol.10 , pp. 1449-1454
    • Kenneson, A.1    Zhang, F.2    Hagedorn, C.H.3
  • 31
    • 0033612144 scopus 로고    scopus 로고
    • FMRP expression as a potential prognostic indicator in fragile X syndrome
    • Tassone F, Hagerman RJ, Iklé DN et al. FMRP expression as a potential prognostic indicator in fragile X syndrome. Am J Med Genet 1999; 84: 250-261.
    • (1999) Am J Med Genet , vol.84 , pp. 250-261
    • Tassone, F.1    Hagerman, R.J.2    Iklé, D.N.3
  • 32
    • 79955428497 scopus 로고    scopus 로고
    • The NCGC pharmaceutical collection: A comprehensive resource of clinically approved drugs enabling repurposing and chemical genomics
    • Huang R, Southall N, Wang Y et al. The NCGC pharmaceutical collection: A comprehensive resource of clinically approved drugs enabling repurposing and chemical genomics. Sci Transl Med 2011; 3: 80ps16.
    • (2011) Sci Transl Med , vol.3 , pp. 80ps16
    • Huang, R.1    Southall, N.2    Wang, Y.3
  • 33
    • 85051691015 scopus 로고    scopus 로고
    • Enabling the large scale analysis of quantitative high throughput screening data
    • Seethala R, Zhang L, eds2nd ed. New York: Taylor & Francis
    • Southall N, Jadhav A, Huang R et al. Enabling the large scale analysis of quantitative high throughput screening dataHandbook of Drug Screening. In: Seethala R, Zhang L, eds2nd ed. New York: Taylor & Francis, 2009442-463.
    • (2009) Handbook of Drug Screening , pp. 442-463
    • Southall, N.1    Jadhav, A.2    Huang, R.3
  • 34
    • 84878805047 scopus 로고    scopus 로고
    • A quantitative homogeneous assay for fragile X mental retardation 1 protein
    • Schutzius G, Bleckmann D, Kapps-Fouthier S et al. A quantitative homogeneous assay for fragile X mental retardation 1 protein. J Neurodev Disord 2013; 5: 8.
    • (2013) J Neurodev Disord , vol.5 , pp. 8
    • Schutzius, G.1    Bleckmann, D.2    Kapps-Fouthier, S.3
  • 35
    • 84862908964 scopus 로고    scopus 로고
    • Inhibition of GSK3b improves hippocampus-dependent learning and rescues neurogenesis in a mouse model of fragile X syndrome
    • GuoW, MurthyAC, Zhang L et al. Inhibition of GSK3b improves hippocampus-dependent learning and rescues neurogenesis in a mouse model of fragile X syndrome. Hum Mol Genet 2012; 21: 681-691.
    • (2012) Hum Mol Genet , vol.21 , pp. 681-691
    • Guo, W.1    Murthy, A.C.2    Zhang, L.3
  • 36
    • 84913586714 scopus 로고    scopus 로고
    • Lithium ameliorates open-field and elevated plus maze behaviors, and brain phospho-glycogen synthase kinase 3-beta expression in fragile X syndrome model mice
    • Chen X, Sun W, Pan Y et al. Lithium ameliorates open-field and elevated plus maze behaviors, and brain phospho-glycogen synthase kinase 3-beta expression in fragile X syndrome model mice. Neurosciences (Riyadh) 2013; 18: 356-362.
    • (2013) Neurosciences (Riyadh) , vol.18 , pp. 356-362
    • Chen, X.1    Sun, W.2    Pan, Y.3
  • 37
    • 79956052512 scopus 로고    scopus 로고
    • Lithium ameliorates phenotypic deficits in a mouse model of fragile X syndrome
    • Liu ZH, Chuang DM, Smith CB. Lithium ameliorates phenotypic deficits in a mouse model of fragile X syndrome. Int J Neuropsychopharmacol 2011; 14: 618-630.
    • (2011) Int J Neuropsychopharmacol , vol.14 , pp. 618-630
    • Liu, Z.H.1    Chuang, D.M.2    Smith, C.B.3
  • 38
    • 0006109789 scopus 로고
    • Activation of purified soluble guanylate cyclase by protoporphyrin IX
    • Ignarro LJ, Wood KS, Wolin MS. Activation of purified soluble guanylate cyclase by protoporphyrin IX. Proc Natl Acad Sci USA 1982; 79: 2870-2873.
    • (1982) Proc Natl Acad Sci USA , vol.79 , pp. 2870-2873
    • Ignarro, L.J.1    Wood, K.S.2    Wolin, M.S.3
  • 39
    • 0017053223 scopus 로고
    • Comparison of the effectiveness of deserpidine, reserpine, and alpha-methyltyrosine on brain biogenic amines
    • Fulton SC, Healy MD. Comparison of the effectiveness of deserpidine, reserpine, and alpha-methyltyrosine on brain biogenic amines. Fed Proc 1976; 35: 2558-2562.
    • (1976) Fed Proc , vol.35 , pp. 2558-2562
    • Fulton, S.C.1    Healy, M.D.2
  • 40
    • 0021999688 scopus 로고
    • Molecular structure of heliomycin, an inhibitor of RNA synthesis
    • Arora SK. Molecular structure of heliomycin, an inhibitor of RNA synthesis. J Antibiot (Tokyo) 1985; 38: 113-115.
    • (1985) J Antibiot (Tokyo) , vol.38 , pp. 113-115
    • Arora, S.K.1
  • 42
    • 0028000251 scopus 로고
    • Inhibition of Helicobacter pylori urease activity by hydroxamic acid derivatives
    • Odake S, Morikawa T, Tsuchiya M et al. Inhibition of Helicobacter pylori urease activity by hydroxamic acid derivatives. Biol Pharm Bull 1994; 17: 1329-1332.
    • (1994) Biol Pharm Bull , vol.17 , pp. 1329-1332
    • Odake, S.1    Morikawa, T.2    Tsuchiya, M.3


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