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Volumn 42, Issue 11, 2014, Pages 7186-7200

DDX6 regulates sequestered nuclear CUG-expanded DMPK-mRNA in dystrophia myotonica type 1

Author keywords

[No Author keywords available]

Indexed keywords

ADENOSINE TRIPHOSPHATE; DEAD BOX PROTEIN; MESSENGER RNA; MUSCLEBLIND LIKE PROTEIN 1; MYOTONIC DYSTROPHY PROTEIN KINASE; NUCLEAR PROTEIN; RIBONUCLEOPROTEIN; UNCLASSIFIED DRUG;

EID: 84903122321     PISSN: 03051048     EISSN: 13624962     Source Type: Journal    
DOI: 10.1093/nar/gku352     Document Type: Article
Times cited : (43)

References (54)
  • 1
    • 70450203364 scopus 로고    scopus 로고
    • Pathogenic mechanisms of myotonic dystrophy
    • Lee, J.E. and Cooper, T.A. (2009) Pathogenic mechanisms of myotonic dystrophy. Biochem.Soc. Trans., 37, 1281-1286.
    • (2009) Biochem.Soc. Trans. , vol.37 , pp. 1281-1286
    • Lee, J.E.1    Cooper, T.A.2
  • 3
    • 84866436425 scopus 로고    scopus 로고
    • The myotonic dystrophies: Molecular, clinical, and therapeutic challenges
    • Udd, B. and Krahe, R. (2012) The myotonic dystrophies: Molecular, clinical, and therapeutic challenges. Lancet Neurol., 11, 891-905.
    • (2012) Lancet Neurol. , vol.11 , pp. 891-905
    • Udd, B.1    Krahe, R.2
  • 4
    • 0026566108 scopus 로고
    • Molecular basis of myotonic dystrophy: Expansion of a trinucleotide (CTG) repeat at the 3' end of a transcript encoding a protein kinase family member
    • Brook, J.D., McCurrach, M.E., Harley, H.G., Buckler, A.J., Church, D., Aburatani, H., Hunter, K., Stanton, V.P., Thirion, J.P., Hudson, T. et al. (1992) Molecular basis of myotonic dystrophy: Expansion of a trinucleotide (CTG) repeat at the 3' end of a transcript encoding a protein kinase family member. Cell, 68, 799-808.
    • (1992) Cell , vol.68 , pp. 799-808
    • Brook, J.D.1    McCurrach, M.E.2    Harley, H.G.3    Buckler, A.J.4    Church, D.5    Aburatani, H.6    Hunter, K.7    Stanton, V.P.8    Thirion, J.P.9    Hudson, T.10
  • 5
  • 9
    • 14044252328 scopus 로고    scopus 로고
    • MBNL1 is the primary determinant of focus formation and aberrant insulin receptor splicing in DM1
    • Dansithong, W., Paul, S., Comai, L. and Reddy, S. (2005) MBNL1 is the primary determinant of focus formation and aberrant insulin receptor splicing in DM1. J. Biol. Chem., 280, 5773-5780.
    • (2005) J. Biol. Chem. , vol.280 , pp. 5773-5780
    • Dansithong, W.1    Paul, S.2    Comai, L.3    Reddy, S.4
  • 11
    • 0035146628 scopus 로고    scopus 로고
    • The CELF family of RNA binding proteins is implicated in cell-specific and developmentally regulated alternative splicing
    • Ladd, A.N., Charlet, N. and Cooper, T.A. (2001) The CELF family of RNA binding proteins is implicated in cell-specific and developmentally regulated alternative splicing. Mol. Cell. Biol., 21, 1285-1296.
    • (2001) Mol. Cell. Biol. , vol.21 , pp. 1285-1296
    • Ladd, A.N.1    Charlet, N.2    Cooper, T.A.3
  • 13
    • 80055097731 scopus 로고    scopus 로고
    • Expanded CUG repeats Dysregulate RNA splicing by altering the stoichiometry of the muscleblind 1 complex
    • Paul, S., Dansithong, W., Jog, S.P., Holt, I., Mittal, S., Brook, J.D., Morris, G.E., Comai, L. and Reddy, S. (2011) Expanded CUG repeats Dysregulate RNA splicing by altering the stoichiometry of the muscleblind 1 complex. J. Biol. Chem., 286, 38427-38438.
    • (2011) J. Biol. Chem. , vol.286 , pp. 38427-38438
    • Paul, S.1    Dansithong, W.2    Jog, S.P.3    Holt, I.4    Mittal, S.5    Brook, J.D.6    Morris, G.E.7    Comai, L.8    Reddy, S.9
  • 14
    • 0032076126 scopus 로고    scopus 로고
    • Disruption of splicing regulated by a CUG-binding protein in myotonic dystrophy
    • Philips, A.V., Timchenko, L.T. and Cooper, T.A. (1998) Disruption of splicing regulated by a CUG-binding protein in myotonic dystrophy. Science, 280, 737-741.
    • (1998) Science , vol.280 , pp. 737-741
    • Philips, A.V.1    Timchenko, L.T.2    Cooper, T.A.3
  • 15
    • 0034873099 scopus 로고    scopus 로고
    • Aberrant regulation of insulin receptor alternative splicing is associated with insulin resistance in myotonic dystrophy
    • Savkur, R.S., Philips, A.V. and Cooper, T.A. (2001) Aberrant regulation of insulin receptor alternative splicing is associated with insulin resistance in myotonic dystrophy. Nat. Genet., 29, 40-47.
    • (2001) Nat. Genet. , vol.29 , pp. 40-47
    • Savkur, R.S.1    Philips, A.V.2    Cooper, T.A.3
  • 17
    • 80055006740 scopus 로고    scopus 로고
    • RNA Foci, CUGBP1, and ZNF9 are the primary targets of the mutant CUG and CCUG repeats expanded in myotonic dystrophies type 1 and type 2
    • Jones, K., Jin, B., Iakova, P., Huichalaf, C., Sarkar, P., Schneider-Gold, C., Schoser, B., Meola, G., Shyu, A.B., Timchenko, N. et al. (2011) RNA Foci, CUGBP1, and ZNF9 are the primary targets of the mutant CUG and CCUG repeats expanded in myotonic dystrophies type 1 and type 2. Am. J. Pathol., 179, 2475-2489.
    • (2011) Am. J. Pathol. , vol.179 , pp. 2475-2489
    • Jones, K.1    Jin, B.2    Iakova, P.3    Huichalaf, C.4    Sarkar, P.5    Schneider-Gold, C.6    Schoser, B.7    Meola, G.8    Shyu, A.B.9    Timchenko, N.10
  • 18
    • 34948834723 scopus 로고    scopus 로고
    • Increased steady-state levels of CUGBP1 in myotonic dystrophy 1 are due to PKC-mediated hyperphosphorylation
    • Kuyumcu-Martinez, N.M., Wang, G.S. and Cooper, T.A. (2007) Increased steady-state levels of CUGBP1 in myotonic dystrophy 1 are due to PKC-mediated hyperphosphorylation. Mol. Cell, 28, 68-78.
    • (2007) Mol. Cell , vol.28 , pp. 68-78
    • Kuyumcu-Martinez, N.M.1    Wang, G.S.2    Cooper, T.A.3
  • 20
    • 77956108781 scopus 로고    scopus 로고
    • CUGBP1 overexpression in mouse skeletal muscle reproduces features of myotonic dystrophy type 1
    • Ward, A.J., Rimer, M., Killian, J.M., Dowling, J.J. and Cooper, T.A. (2010) CUGBP1 overexpression in mouse skeletal muscle reproduces features of myotonic dystrophy type 1. Hum. Mol. Genet., 19, 3614-3622.
    • (2010) Hum. Mol. Genet. , vol.19 , pp. 3614-3622
    • Ward, A.J.1    Rimer, M.2    Killian, J.M.3    Dowling, J.J.4    Cooper, T.A.5
  • 21
    • 0030841672 scopus 로고    scopus 로고
    • Expansion of a CUG trinucleotide repeat in the 3' untranslated region of myotonic dystrophy protein kinase transcripts results in nuclear retention of transcripts
    • Davis, B.M., McCurrach, M.E., Taneja, K.L., Singer, R.H. and Housman, D.E. (1997) Expansion of a CUG trinucleotide repeat in the 3' untranslated region of myotonic dystrophy protein kinase transcripts results in nuclear retention of transcripts. Proc. Natl. Acad. Sci. U.S.A., 94, 7388-7393.
    • (1997) Proc. Natl. Acad. Sci. U.S.A. , vol.94 , pp. 7388-7393
    • Davis, B.M.1    McCurrach, M.E.2    Taneja, K.L.3    Singer, R.H.4    Housman, D.E.5
  • 22
    • 0035394801 scopus 로고    scopus 로고
    • In vivo co-localisation of MBNL protein with DMPK expanded-repeat transcripts
    • Fardaei, M., Larkin, K., Brook, J.D. and Hamshere, M.G. (2001) In vivo co-localisation of MBNL protein with DMPK expanded-repeat transcripts. Nucleic Acids Res., 29, 2766-2771.
    • (2001) Nucleic Acids Res. , vol.29 , pp. 2766-2771
    • Fardaei, M.1    Larkin, K.2    Brook, J.D.3    Hamshere, M.G.4
  • 23
    • 0036537492 scopus 로고    scopus 로고
    • Three proteins, MBNL, MBLL and MBXL, co-localize in vivo with nuclear foci of expanded-repeat transcripts in DM1 and DM2 cells
    • Fardaei, M., Rogers, M.T., Thorpe, H.M., Larkin, K., Hamshere, M.G., Harper, P.S. and Brook, J.D. (2002) Three proteins, MBNL, MBLL and MBXL, co-localize in vivo with nuclear foci of expanded-repeat transcripts in DM1 and DM2 cells. Hum. Mol. Genet., 11, 805-814.
    • (2002) Hum. Mol. Genet. , vol.11 , pp. 805-814
    • Fardaei, M.1    Rogers, M.T.2    Thorpe, H.M.3    Larkin, K.4    Hamshere, M.G.5    Harper, P.S.6    Brook, J.D.7
  • 24
    • 0031953529 scopus 로고    scopus 로고
    • Localization of trinucleotide repeat sequences in myotonic dystrophy cells using a single fluorochrome-labeled PNA probe
    • Taneja, K.L. (1998) Localization of trinucleotide repeat sequences in myotonic dystrophy cells using a single fluorochrome-labeled PNA probe. Biotechniques, 24, 472-476.
    • (1998) Biotechniques , vol.24 , pp. 472-476
    • Taneja, K.L.1
  • 25
    • 0028947317 scopus 로고
    • Foci of trinucleotide repeat transcripts in nuclei of myotonic dystrophy cells and tissues
    • Taneja, K.L., McCurrach, M., Schalling, M., Housman, D. and Singer, R.H. (1995) Foci of trinucleotide repeat transcripts in nuclei of myotonic dystrophy cells and tissues. J. Cell Biol., 128, 995-1002.
    • (1995) J. Cell Biol. , vol.128 , pp. 995-1002
    • Taneja, K.L.1    McCurrach, M.2    Schalling, M.3    Housman, D.4    Singer, R.H.5
  • 29
    • 84875171510 scopus 로고    scopus 로고
    • Toward a molecular understanding of RNA remodeling by DEAD-box proteins
    • Russell, R., Jarmoskaite, I. and Lambowitz, A.M. (2013) Toward a molecular understanding of RNA remodeling by DEAD-box proteins. RNA Biol., 10, 44-55.
    • (2013) RNA Biol. , vol.10 , pp. 44-55
    • Russell, R.1    Jarmoskaite, I.2    Lambowitz, A.M.3
  • 30
    • 33745159015 scopus 로고    scopus 로고
    • Xp54 and related (DDX6-like) RNA helicases: Roles in messenger RNP assembly, translation regulation and RNA degradation
    • Weston, A. and Sommerville, J. (2006) Xp54 and related (DDX6-like) RNA helicases: Roles in messenger RNP assembly, translation regulation and RNA degradation. Nucleic Acids Res., 34, 3082-3094.
    • (2006) Nucleic Acids Res. , vol.34 , pp. 3082-3094
    • Weston, A.1    Sommerville, J.2
  • 31
    • 56849103665 scopus 로고    scopus 로고
    • The control of mRNA decapping and P-body formation
    • Franks, T.M. and Lykke-Andersen, J. (2008) The control of mRNA decapping and P-body formation. Mol. Cell, 32, 605-615.
    • (2008) Mol. Cell , vol.32 , pp. 605-615
    • Franks, T.M.1    Lykke-Andersen, J.2
  • 32
    • 65649125183 scopus 로고    scopus 로고
    • Role of p54 RNA helicase activity and its C-terminal domain in translational repression, P-body localization and assembly
    • Minshall, N., Kress, M., Weil, D. and Standart, N. (2009) Role of p54 RNA helicase activity and its C-terminal domain in translational repression, P-body localization and assembly. Mol. Biol. Cell, 20, 2464-2472.
    • (2009) Mol. Biol. Cell , vol.20 , pp. 2464-2472
    • Minshall, N.1    Kress, M.2    Weil, D.3    Standart, N.4
  • 33
    • 33745894330 scopus 로고    scopus 로고
    • Translation repression in human cells by microRNA-induced gene silencing requires RCK/p54
    • Chu, C.Y. and Rana, T.M. (2006) Translation repression in human cells by microRNA-induced gene silencing requires RCK/p54. PLoS Biol., 4, e210.
    • (2006) PLoS Biol. , vol.4
    • Chu, C.Y.1    Rana, T.M.2
  • 34
    • 29144481702 scopus 로고    scopus 로고
    • Multiple processing body factors and the ARE binding protein TTP activate mRNA decapping
    • Fenger-Gron, M., Fillman, C., Norrild, B. and Lykke-Andersen, J. (2005) Multiple processing body factors and the ARE binding protein TTP activate mRNA decapping. Mol. Cell, 20, 905-915.
    • (2005) Mol. Cell , vol.20 , pp. 905-915
    • Fenger-Gron, M.1    Fillman, C.2    Norrild, B.3    Lykke-Andersen, J.4
  • 35
    • 77952628837 scopus 로고    scopus 로고
    • Regulation of translation by stress granules and processing bodies
    • Kedersha, N. and Anderson, P. (2009) Regulation of translation by stress granules and processing bodies. Prog. Mol. Biol. Transl. Sci., 90, 155-185.
    • (2009) Prog. Mol. Biol. Transl. Sci. , vol.90 , pp. 155-185
    • Kedersha, N.1    Anderson, P.2
  • 36
    • 17644383664 scopus 로고    scopus 로고
    • Lentiviral-mediated silencing of SOD1 through RNA interference retards disease onset and progression in a mouse model of ALS
    • Raoul, C., Abbas-Terki, T., Bensadoun, J.C., Guillot, S., Haase, G., Szulc, J., Henderson, C.E. and Aebischer, P. (2005) Lentiviral-mediated silencing of SOD1 through RNA interference retards disease onset and progression in a mouse model of ALS. Nat. Med., 11, 423-428.
    • (2005) Nat. Med. , vol.11 , pp. 423-428
    • Raoul, C.1    Abbas-Terki, T.2    Bensadoun, J.C.3    Guillot, S.4    Haase, G.5    Szulc, J.6    Henderson, C.E.7    Aebischer, P.8
  • 37
    • 80053625750 scopus 로고    scopus 로고
    • Translational coregulation of 5'TOP mRNAs by TIA-1 and TIAR
    • Damgaard, C.K. and Lykke-Andersen, J. (2011) Translational coregulation of 5'TOP mRNAs by TIA-1 and TIAR. Genes Dev., 25, 2057-2068.
    • (2011) Genes Dev. , vol.25 , pp. 2057-2068
    • Damgaard, C.K.1    Lykke-Andersen, J.2
  • 38
    • 33744762160 scopus 로고    scopus 로고
    • DM2 intronic expansions: Evidence for CCUG accumulation without flanking sequence or effects on ZNF9 mRNA processing or protein expression
    • Margolis, J.M., Schoser, B.G., Moseley, M.L., Day, J.W. and Ranum, L.P. (2006) DM2 intronic expansions: Evidence for CCUG accumulation without flanking sequence or effects on ZNF9 mRNA processing or protein expression. Hum. Mol. Genet., 15, 1808-1815.
    • (2006) Hum. Mol. Genet. , vol.15 , pp. 1808-1815
    • Margolis, J.M.1    Schoser, B.G.2    Moseley, M.L.3    Day, J.W.4    Ranum, L.P.5
  • 41
    • 0036849108 scopus 로고    scopus 로고
    • HnRNP A1 controls HIV-1 mRNA splicing through cooperative binding to intron and exon splicing silencers in the context of a conserved secondary structure
    • Damgaard, C.K., Tange, T.O. and Kjems, J. (2002) hnRNP A1 controls HIV-1 mRNA splicing through cooperative binding to intron and exon splicing silencers in the context of a conserved secondary structure. RNA, 8, 1401-1415.
    • (2002) RNA , vol.8 , pp. 1401-1415
    • Damgaard, C.K.1    Tange, T.O.2    Kjems, J.3
  • 42
    • 0035445656 scopus 로고    scopus 로고
    • The myotonic dystrophy expanded CUG repeat tract is necessary but not sufficient to disrupt C2C12 myoblast differentiation
    • Amack, J.D. and Mahadevan, M.S. (2001) The myotonic dystrophy expanded CUG repeat tract is necessary but not sufficient to disrupt C2C12 myoblast differentiation. Hum. Mol. Genet., 10, 1879-1887.
    • (2001) Hum. Mol. Genet. , vol.10 , pp. 1879-1887
    • Amack, J.D.1    Mahadevan, M.S.2
  • 44
    • 33947301293 scopus 로고    scopus 로고
    • TTP and BRF proteins nucleate processing body formation to silence mRNAs with AU-rich elements
    • Franks, T.M. and Lykke-Andersen, J. (2007) TTP and BRF proteins nucleate processing body formation to silence mRNAs with AU-rich elements. Genes Dev., 21, 719-735.
    • (2007) Genes Dev. , vol.21 , pp. 719-735
    • Franks, T.M.1    Lykke-Andersen, J.2
  • 45
    • 17844371700 scopus 로고    scopus 로고
    • A role for eIF4E and eIF4E-transporter in targeting mRNPs to mammalian processing bodies
    • Andrei, M.A., Ingelfinger, D., Heintzmann, R., Achsel, T., Rivera-Pomar, R. and Luhrmann, R. (2005) A role for eIF4E and eIF4E-transporter in targeting mRNPs to mammalian processing bodies. RNA, 11, 717-727.
    • (2005) RNA , vol.11 , pp. 717-727
    • Andrei, M.A.1    Ingelfinger, D.2    Heintzmann, R.3    Achsel, T.4    Rivera-Pomar, R.5    Luhrmann, R.6
  • 47
    • 81055148308 scopus 로고    scopus 로고
    • Myoblasts generated by lentiviral mediated MyoD transduction of myotonic dystrophy type 1 (DM1) fibroblasts can be used for assays of therapeutic molecules
    • Larsen, J., Pettersson, O.J., Jakobsen, M., Thomsen, R., Pedersen, C.B., Hertz, J.M., Gregersen, N., Corydon, T.J. and Jensen, T.G. (2011) Myoblasts generated by lentiviral mediated MyoD transduction of myotonic dystrophy type 1 (DM1) fibroblasts can be used for assays of therapeutic molecules. BMC Res. Notes, 4, 490-498.
    • (2011) BMC Res. Notes , vol.4 , pp. 490-498
    • Larsen, J.1    Pettersson, O.J.2    Jakobsen, M.3    Thomsen, R.4    Pedersen, C.B.5    Hertz, J.M.6    Gregersen, N.7    Corydon, T.J.8    Jensen, T.G.9
  • 48
    • 79961016813 scopus 로고    scopus 로고
    • Intermolecular interactions within the abundant DEAD-box protein Dhh1 regulate its activity in vivo
    • Dutta, A., Zheng, S., Jain, D., Cameron, C.E. and Reese, J.C. (2011) Intermolecular interactions within the abundant DEAD-box protein Dhh1 regulate its activity in vivo. J. Biol. Chem., 286, 27454-27470.
    • (2011) J. Biol. Chem. , vol.286 , pp. 27454-27470
    • Dutta, A.1    Zheng, S.2    Jain, D.3    Cameron, C.E.4    Reese, J.C.5
  • 49
    • 84867064003 scopus 로고    scopus 로고
    • Structural basis for RNA-duplex recognition and unwinding by the DEAD-box helicase Mss116p
    • Mallam, A.L., Del Campo, M., Gilman, B., Sidote, D.J. and Lambowitz, A.M. (2012) Structural basis for RNA-duplex recognition and unwinding by the DEAD-box helicase Mss116p. Nature, 490, 121-125.
    • (2012) Nature , vol.490 , pp. 121-125
    • Mallam, A.L.1    Del Campo, M.2    Gilman, B.3    Sidote, D.J.4    Lambowitz, A.M.5
  • 50
    • 25144482816 scopus 로고    scopus 로고
    • General translational repression by activators of mRNA decapping
    • Coller, J. and Parker, R. (2005) General translational repression by activators of mRNA decapping. Cell, 122, 875-886.
    • (2005) Cell , vol.122 , pp. 875-886
    • Coller, J.1    Parker, R.2
  • 51
    • 0035674477 scopus 로고    scopus 로고
    • The DEAD box helicase, Dhh1p, functions in mRNA decapping and interacts with both the decapping and deadenylase complexes
    • Coller, J.M., Tucker, M., Sheth, U., Valencia-Sanchez, M.A. and Parker, R. (2001) The DEAD box helicase, Dhh1p, functions in mRNA decapping and interacts with both the decapping and deadenylase complexes. RNA, 7, 1717-1727.
    • (2001) RNA , vol.7 , pp. 1717-1727
    • Coller, J.M.1    Tucker, M.2    Sheth, U.3    Valencia-Sanchez, M.A.4    Parker, R.5
  • 52
    • 0037081724 scopus 로고    scopus 로고
    • RNA helicase p54 (DDX6) is a shuttling protein involved in nuclear assembly of stored mRNP particles
    • Smillie, D.A. and Sommerville, J. (2002) RNA helicase p54 (DDX6) is a shuttling protein involved in nuclear assembly of stored mRNP particles. J. Cell Sci., 115, 395-407.
    • (2002) J. Cell Sci. , vol.115 , pp. 395-407
    • Smillie, D.A.1    Sommerville, J.2
  • 53
    • 34548441225 scopus 로고    scopus 로고
    • Defective mRNA in myotonic dystrophy accumulates at the periphery of nuclear splicing speckles
    • Holt, I., Mittal, S., Furling, D., Butler-Browne, G.S., Brook, J.D. and Morris, G.E. (2007) Defective mRNA in myotonic dystrophy accumulates at the periphery of nuclear splicing speckles. Genes Cell, 12, 1035-1048.
    • (2007) Genes Cell , vol.12 , pp. 1035-1048
    • Holt, I.1    Mittal, S.2    Furling, D.3    Butler-Browne, G.S.4    Brook, J.D.5    Morris, G.E.6
  • 54
    • 0035671046 scopus 로고    scopus 로고
    • A conserved role of a DEAD box helicase in mRNA masking
    • Minshall, N., Thom, G. and Standart, N. (2001) A conserved role of a DEAD box helicase in mRNA masking. RNA, 7, 1728-1742
    • (2001) RNA , vol.7 , pp. 1728-1742
    • Minshall, N.1    Thom, G.2    Standart, N.3


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