CELF1

Summary

Gene Symbol: CELF1
Description: CUGBP Elav-like family member 1
Alias: BRUNOL2, CUG-BP, CUGBP, CUGBP1, EDEN-BP, NAB50, NAPOR, hNab50, CUGBP Elav-like family member 1, 50 kDa nuclear polyadenylated RNA-binding protein, CELF-1, CUG RNA-binding protein, CUG triplet repeat RNA-binding protein 1, CUG-BP- and ETR-3-like factor 1, CUG-BP1, EDEN-BP homolog, RNA-binding protein BRUNOL-2, bruno-like 2, bruno-like protein 2, deadenylation factor CUG-BP, embryo deadenylation element binding protein, embryo deadenylation element-binding protein homolog, nuclear polyadenylated RNA-binding protein, 50-kD
Species: human

Top Publications

  1. ncbi RNA CUG repeats sequester CUGBP1 and alter protein levels and activity of CUGBP1
    N A Timchenko
    Department of Pathology and Huffington Center on Aging, Baylor College of Medicine, Houston, Texas 77030, USA
    J Biol Chem 276:7820-6. 2001
  2. ncbi CUGBP1 is required for IFNbeta-mediated induction of dominant-negative CEBPbeta and suppression of SIV replication in macrophages
    Justyna M Dudaronek
    Department of Molecular and Comparative Pathobiology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
    J Immunol 179:7262-9. 2007
  3. pmc Ectopic expression of cyclin D3 corrects differentiation of DM1 myoblasts through activation of RNA CUG-binding protein, CUGBP1
    Elizabeth Salisbury
    Department of Pathology, Huffington Center on Aging, Baylor College of Medicine, Houston, TX 77030, USA
    Exp Cell Res 314:2266-78. 2008
  4. pmc HDAC1 promotes liver proliferation in young mice via interactions with C/EBPbeta
    Guo Li Wang
    Huffington Center on Aging and Department of Pathology, Department of Medicine, and Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, Texas 77030, USA
    J Biol Chem 283:26179-87. 2008
  5. pmc Structural insights into RNA recognition by the alternate-splicing regulator CUG-binding protein 1
    Marianna Teplova
    Structural Biology Program, Memorial Sloan Kettering Cancer Center, New York, NY 10021, USA
    Structure 18:1364-77. 2010
  6. ncbi Altered expression of CUG binding protein 1 mRNA in myotonic dystrophy 1: possible RNA-RNA interaction
    Tomoji Watanabe
    Department of Neurology, Toranomon Hospital and Okinaka Memorial Institute for Medical Research, 222 Toranomon, Minato ku, Tokyo 105 0001, Japan
    Neurosci Res 49:47-54. 2004
  7. pmc Identification of a (CUG)n triplet repeat RNA-binding protein and its expression in myotonic dystrophy
    L T Timchenko
    Department of Medicine, Baylor College of Medicine, Houston, TX 77030, USA
    Nucleic Acids Res 24:4407-14. 1996
  8. pmc CUG repeat binding protein (CUGBP1) interacts with the 5' region of C/EBPbeta mRNA and regulates translation of C/EBPbeta isoforms
    N A Timchenko
    Huffington Center on Aging, Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
    Nucleic Acids Res 27:4517-25. 1999
  9. pmc Molecular basis for impaired muscle differentiation in myotonic dystrophy
    N A Timchenko
    Huffington Center on Aging, Baylor College of Medicine, Houston, Texas 77030, USA
    Mol Cell Biol 21:6927-38. 2001
  10. ncbi MBNL1 is the primary determinant of focus formation and aberrant insulin receptor splicing in DM1
    Warunee Dansithong
    Department of Biochemistry and Molecular Biology, Institute for Genetic Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033, USA
    J Biol Chem 280:5773-80. 2005

Research Grants

  1. Molecular mechanisms of Myotonic Dystrophy 1
    Lubov T Timchenko; Fiscal Year: 2012
  2. Thomas A Cooper; Fiscal Year: 2016
  3. Regulation of Cell Growth by RNA Binding Proteins
    Nikolai A Timchenko; Fiscal Year: 2012
  4. Genetic Regulation of Complex Neurological Disease
    Wayne N Frankel; Fiscal Year: 2012
  5. Genetic Regulation of Complex Neurological Diseases
    Wayne N Frankel; Fiscal Year: 2013
  6. Andrew Berglund; Fiscal Year: 2014
  7. The toxicity of the RNA CGG repeats in FXTAS
    Lubov T Timchenko; Fiscal Year: 2013
  8. Maha Abdellatif; Fiscal Year: 2014
  9. Eric T Wang; Fiscal Year: 2016
  10. MAURICE SCOTT SWANSON; Fiscal Year: 2014

Scientific Experts

Detail Information

Publications142 found, 100 shown here

  1. ncbi RNA CUG repeats sequester CUGBP1 and alter protein levels and activity of CUGBP1
    N A Timchenko
    Department of Pathology and Huffington Center on Aging, Baylor College of Medicine, Houston, Texas 77030, USA
    J Biol Chem 276:7820-6. 2001
    An RNA CUG triplet repeat binding protein, CUGBP1, regulates splicing and translation of various RNAs...
  2. ncbi CUGBP1 is required for IFNbeta-mediated induction of dominant-negative CEBPbeta and suppression of SIV replication in macrophages
    Justyna M Dudaronek
    Department of Molecular and Comparative Pathobiology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
    J Immunol 179:7262-9. 2007
    ..Alternative translation of C/EBPbeta mRNA and increased production of LIP can be mediated by CUGBP1 (CUG-repeat RNA-binding protein 1)...
  3. pmc Ectopic expression of cyclin D3 corrects differentiation of DM1 myoblasts through activation of RNA CUG-binding protein, CUGBP1
    Elizabeth Salisbury
    Department of Pathology, Huffington Center on Aging, Baylor College of Medicine, Houston, TX 77030, USA
    Exp Cell Res 314:2266-78. 2008
    ..Differentiation of myocytes is impaired in patients with myotonic dystrophy type 1, DM1. CUG repeat binding protein, CUGBP1, is a key regulator of translation of proteins that are involved in muscle development and differentiation...
  4. pmc HDAC1 promotes liver proliferation in young mice via interactions with C/EBPbeta
    Guo Li Wang
    Huffington Center on Aging and Department of Pathology, Department of Medicine, and Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, Texas 77030, USA
    J Biol Chem 283:26179-87. 2008
    ..Consistent with the positive role of HDAC1-C/EBPbeta complexes in liver proliferation, we have found that the CUGBP1-HDAC1-C/EBPbeta pathway is activated in human tumor liver samples, suggesting that HDAC1-C/EBPbeta complexes are ..
  5. pmc Structural insights into RNA recognition by the alternate-splicing regulator CUG-binding protein 1
    Marianna Teplova
    Structural Biology Program, Memorial Sloan Kettering Cancer Center, New York, NY 10021, USA
    Structure 18:1364-77. 2010
    CUG-binding protein 1 (CUGBP1) regulates multiple aspects of nuclear and cytoplasmic mRNA processing, with implications for onset of myotonic dystrophy. CUGBP1 harbors three RRM domains and preferentially targets UGU-rich mRNA elements...
  6. ncbi Altered expression of CUG binding protein 1 mRNA in myotonic dystrophy 1: possible RNA-RNA interaction
    Tomoji Watanabe
    Department of Neurology, Toranomon Hospital and Okinaka Memorial Institute for Medical Research, 222 Toranomon, Minato ku, Tokyo 105 0001, Japan
    Neurosci Res 49:47-54. 2004
    ..results using differential display analysis, the present study focused on the expression of CUG binding protein 1 (CUGBP1) mRNA...
  7. pmc Identification of a (CUG)n triplet repeat RNA-binding protein and its expression in myotonic dystrophy
    L T Timchenko
    Department of Medicine, Baylor College of Medicine, Houston, TX 77030, USA
    Nucleic Acids Res 24:4407-14. 1996
    ..CUG-BP1 and CUG-BP2 have been identified as isoforms of a novel heterogeneous nuclear ribonucleoprotein (hnRNP), hNab50. The CUG-BP/hNab50 protein is localized predominantly in the nucleus and is associated with polyadenylated RNAs in ..
  8. pmc CUG repeat binding protein (CUGBP1) interacts with the 5' region of C/EBPbeta mRNA and regulates translation of C/EBPbeta isoforms
    N A Timchenko
    Huffington Center on Aging, Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
    Nucleic Acids Res 27:4517-25. 1999
    ..Here we present evidence that CUG repeat binding protein, CUGBP1, interacts with the 5' region of C/EBPbeta mRNA and regulates translation of C/EBPbeta isoforms...
  9. pmc Molecular basis for impaired muscle differentiation in myotonic dystrophy
    N A Timchenko
    Huffington Center on Aging, Baylor College of Medicine, Houston, Texas 77030, USA
    Mol Cell Biol 21:6927-38. 2001
    ..Skeletal muscle cells from DM patients fail to induce cytoplasmic levels of a CUG RNA binding protein, CUGBP1, while normal differentiated cells accumulate CUGBP1 in the cytoplasm...
  10. ncbi MBNL1 is the primary determinant of focus formation and aberrant insulin receptor splicing in DM1
    Warunee Dansithong
    Department of Biochemistry and Molecular Biology, Institute for Genetic Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033, USA
    J Biol Chem 280:5773-80. 2005
    ..The data therefore support MBNL1-mediated therapy for DM1...
  11. pmc CUG-BP binds to RNA substrates and recruits PARN deadenylase
    Karen C M Moraes
    Department of Microbiology, Immunology and Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, 80523, USA
    RNA 12:1084-91. 2006
    ..CUG-BP, therefore, is the first RNA-binding protein shown to directly recruit a deadenylase to an RNA substrate...
  12. pmc C/EBPbeta suppression by interruption of CUGBP1 resulting from a complex rearrangement of MLL
    William T Choi
    Division of Pediatrics, Cell Therapy Section, University of Texas M D Anderson Cancer Center, 1515 Holcombe Boulevard, Pediatrics Research Unit 853, Houston, TX 77030 4009, USA
    Cancer Genet Cytogenet 177:108-14. 2007
    ..We show that this latter event interrupts the CUG triplet repeat binding protein-1 (CUGBP1) gene, a translational enhancer of C/EBPbeta...
  13. pmc Increased steady-state levels of CUGBP1 in myotonic dystrophy 1 are due to PKC-mediated hyperphosphorylation
    N Muge Kuyumcu-Martinez
    Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
    Mol Cell 28:68-78. 2007
    ..transcripts accumulate in nuclei and alter the functions of RNA-binding proteins such as CUG-binding protein 1 (CUGBP1). CUGBP1 levels are increased in DM1 myoblasts, heart, and skeletal muscle tissues and in some DM1 mouse models...
  14. ncbi Quantitative analysis of CUG-BP1 binding to RNA repeats
    Daisuke Mori
    Department of Life Sciences, Graduate School of Arts and Sciences, The University of Tokyo, 3 8 1 Komaba, Meguro ku, Tokyo, Japan
    J Biochem 143:377-83. 2008
    ..This is the first report of a binding constant for CUG-BP1 calculated in vitro...
  15. doi Dual localization of the RNA binding protein CUGBP-1 to stress granule and perinucleolar compartment
    Ken Fujimura
    Department of Life Sciences, Graduate School of Liberal Arts and Sciences, University of Tokyo, Tokyo, 3 8 1 Komaba, Meguro ku, 153 8902, Japan
    Exp Cell Res 314:543-53. 2008
    The mRNA-binding protein CUGBP-1 is a multi-faceted factor, involved in a wide range of biological processes including splicing, translation initiation and mRNA degradation...
  16. pmc Conserved GU-rich elements mediate mRNA decay by binding to CUG-binding protein 1
    Irina A Vlasova
    Center for Infectious Diseases and Microbiology Translational Research, University of Minnesota, Minneapolis, MN 55455, USA
    Mol Cell 29:263-70. 2008
    ..CUG-binding protein 1 (CUGBP1) was identified as the major GRE-binding activity in cytoplasmic extracts from primary human T cells based on ..
  17. pmc SRp20 and CUG-BP1 modulate insulin receptor exon 11 alternative splicing
    Supriya Sen
    VA San Diego Healthcare System, San Diego, California 92161, USA
    Mol Cell Biol 29:871-80. 2009
    ..Thus, we propose that SRp20, SF2/ASF, and CUG-BP1 act antagonistically to regulate IR alternative splicing in vivo and that the relative ratios of SRp20 and SF2/ASF to CUG-BP1 in different cells determine the degree of exon inclusion...
  18. pmc Structural basis for the sequence-specific RNA-recognition mechanism of human CUG-BP1 RRM3
    Kengo Tsuda
    RIKEN Systems and Structural Biology Center, Tsurumi, Japan
    Nucleic Acids Res 37:5151-66. 2009
    ....
  19. pmc Analysis of CUGBP1 targets identifies GU-repeat sequences that mediate rapid mRNA decay
    Bernd Rattenbacher
    Center for Infectious Diseases and Microbiology Translational Research, Department of Microbiology, University of Minnesota, Minneapolis, MN 55455, USA
    Mol Cell Biol 30:3970-80. 2010
    CUG-repeat binding protein 1 (CUGBP1) mediates selective mRNA decay by binding to GU-rich elements (GREs) containing the sequence UGUUUGUUUGU found in the 3' untranslated region (UTR) of short-lived transcripts...
  20. pmc CUGBP1 overexpression in mouse skeletal muscle reproduces features of myotonic dystrophy type 1
    Amanda J Ward
    Department of Pathology and Immunology, Baylor College of Medicine, Houston, TX 77030, USA
    Hum Mol Genet 19:3614-22. 2010
    ..nuclear depletion of Muscleblind-like 1 (MBNL1) and to the increased steady-state levels of CUG-binding protein 1 (CUGBP1)...
  21. pmc p21(WAF1/CIP1) upregulation through the stress granule-associated protein CUGBP1 confers resistance to bortezomib-mediated apoptosis
    Cristina Gareau
    Department of Molecular Biology, Medical Biochemistry, and Pathology, Faculty of Medicine, Laval University, CHUQ Research Centre St François d Assise Research Centre CRCHUQ CRSFA, Quebec, Canada
    PLoS ONE 6:e20254. 2011
    ..The mechanism underlying p21 mRNA stabilization, however, remains unknown...
  22. pmc Regulation of CUG-binding protein 1 (CUGBP1) binding to target transcripts upon T cell activation
    Daniel Beisang
    Center for Infectious Diseases and Microbiology Translational Research, University of Minnesota, Minneapolis, Minnesota 55455, USA
    J Biol Chem 287:950-60. 2012
    The RNA-binding protein, CUG-binding protein 1 (CUGBP1), regulates gene expression at the levels of alternative splicing, mRNA degradation, and translation...
  23. pmc The RNA-binding protein CUG-BP1 increases survivin expression in oesophageal cancer cells through enhanced mRNA stability
    Elizabeth T Chang
    Cell Biology Group, Department of Surgery, University of Maryland School of Medicine, Baltimore, MD 21201, USA
    Biochem J 446:113-23. 2012
    ..Co-transfection experiments with small interfering RNA directed against survivin suggest that the anti-apoptotic role for CUG-BP1 is not entirely dependent on its effect on survivin expression...
  24. pmc Competition of CUGBP1 and calreticulin for the regulation of p21 translation determines cell fate
    Polina Iakova
    Department of Pathology and Huffington Center on Aging, Houston, TX, USA
    EMBO J 23:406-17. 2004
    ..We found that RNA-binding proteins are critical regulators of p21 during senescence. Two RNA-binding proteins, CUGBP1 and calreticulin (CRT), interact with the same nucleotide sequences within the 5' region of p21 mRNA, but have ..
  25. pmc Calreticulin interacts with C/EBPalpha and C/EBPbeta mRNAs and represses translation of C/EBP proteins
    Lubov T Timchenko
    Department of Pathology and Huffington Center on Aging Departments of Medicine and Molecular Physiology and Biophysics, Baylor College of Medicine, Houston, Texas 77030, USA
    Mol Cell Biol 22:7242-57. 2002
    We previously identified an RNA binding protein, CUGBP1, which binds to GCN repeats located within the 5' region of C/EBPbeta mRNAs and regulates translation of C/EBPbeta isoforms...
  26. pmc Region-specific alternative splicing in the nervous system: implications for regulation by the RNA-binding protein NAPOR
    Wenqing Zhang
    Howard Hughes Medical Institute and Department of Biological Sciences, University of Pittsburgh, Pennsylvania 15260, USA
    RNA 8:671-85. 2002
    ..the biochemical properties and spatial expression of mouse neuroblastoma apoptosis-related RNA-binding protein (NAPOR; also called NAPOR-1) are consistent with its roles in the regulation of the exon 5 and exon 21 splicing events of ..
  27. ncbi Expression, localization and tau exon 10 splicing activity of the brain RNA-binding protein TNRC4
    J Paul Chapple
    MRC Centre for Neurodegeneration Research, Institute of Psychiatry, King s College London, De Crespigny Park, London SE5 8AF, UK
    Hum Mol Genet 16:2760-9. 2007
    ..This study represents the first characterization of TNRC4 and provides further insight into the mechanisms of brain-specific alternative splicing and their possible pathological implications...
  28. pmc Mechanism of the internal ribosome entry site-mediated translation of serine hydroxymethyltransferase 1
    Jennifer T Fox
    Graduate Field of Biochemistry and Molecular and Cellular Biology, Cornell University, Ithaca, New York 14853, USA
    J Biol Chem 284:31085-96. 2009
    ..Depletion of the SHMT1 IRES-specific trans-acting factor (ITAF) CUG-binding protein 1 (CUGBP1) from in vitro translation extracts or deletion of the CUGBP1 binding site on the 3'-UTR of the SHMT1 transcript ..
  29. pmc PKC inhibition ameliorates the cardiac phenotype in a mouse model of myotonic dystrophy type 1
    Guey Shin Wang
    Department of Pathology, Baylor College of Medicine, Houston, Texas, USA
    J Clin Invest 119:3797-806. 2009
    ..due to disrupted functioning of the splicing regulators muscleblind-like 1 (MBNL1) and CUG-binding protein 1 (CUGBP1)...
  30. pmc Expansion of CUG RNA repeats causes stress and inhibition of translation in myotonic dystrophy 1 (DM1) cells
    Claudia Huichalaf
    Department of Molecular Physiology and Biophysics, Baylor College of Medicine, 1 Baylor Plaza, Houston, TX 77030, USA
    FASEB J 24:3706-19. 2010
    ..we performed HPLC-based purification of stress granules (SGs), immunoanalysis of SGs with stress markers TIA-1, CUGBP1, and ph-eIF2, site-specific mutagenesis, and examinations of RNA-protein and protein-protein interactions in ..
  31. ncbi GSK3beta-cyclin D3-CUGBP1-eIF2 pathway in aging and in myotonic dystrophy
    Jingling Jin
    Huffington Center on Aging and Departments of Pathology and Molecular Physiology and Biophysics, Baylor College of Medicine, Houston, TX, USA
    Cell Cycle 8:2356-9. 2009
    ..livers of old mice leads to the accumulation of a transcriptional C/EBPalpha-HDAC1-Brm complex and translational CUGBP1-eIF2 complex...
  32. pmc Altered RNA splicing contributes to skeletal muscle pathology in Kennedy disease knock-in mice
    Zhigang Yu
    Department of Pathology, The University of Michigan Medical School, Ann Arbor, MI 48109, USA
    Dis Model Mech 2:500-7. 2009
    ..Changes in RNA splicing are associated with increased expression of the RNA-binding protein CUGBP1. Furthermore, we show that skeletal muscle denervation in the absence of a repeat expansion leads to increased ..
  33. pmc Expression of 24,426 human alternative splicing events and predicted cis regulation in 48 tissues and cell lines
    John C Castle
    Rosetta Inpharmatics LLC, Seattle, WA 98109, USA
    Nat Genet 40:1416-25. 2008
    ..UCUCU and UGCAUG seem to have similar function but independent action, occurring 5' and 3', respectively, of 33% of the cassette exons upregulated in skeletal muscle but co-occurring for only 2%...
  34. pmc The RNA-binding protein CUGBP1 regulates stability of tumor necrosis factor mRNA in muscle cells: implications for myotonic dystrophy
    Libin Zhang
    Department of Microbiology, Immunology, and Pathology, Colorado State University, Fort Collins, Colorado 80523, USA
    J Biol Chem 283:22457-63. 2008
    ..of a toxic RNA from the mutant allele, which interferes with function of several RNA-binding proteins, including CUGBP1. Here we show that expression of a mutant DMPK 3'-UTR containing 960 CUG repeats is sufficient to increase ..
  35. doi CELF-mediated alternative splicing is required for cardiac function during early, but not later, postnatal life
    Fulvia Terenzi
    Department of Cell Biology, Lerner Research Institute, Cleveland Clinic Foundation, 9500 Euclid Ave NC10, Cleveland, OH 44195, USA
    J Mol Cell Cardiol 46:395-404. 2009
    ..Our results suggest that CELF proteins are key players in the integrated gene expression program involved in postnatal cardiac remodeling and maturation...
  36. pmc MBNL and CELF proteins regulate alternative splicing of the skeletal muscle chloride channel CLCN1
    Yoshihiro Kino
    Laboratory for Structural Neuropathology, RIKEN Brain Science Institute, Wako Shi, Saitama 351 0198, Japan
    Nucleic Acids Res 37:6477-90. 2009
    ..Collectively, these results provide a mechanistic model for the regulation of Clcn1 splicing, and reveal novel regulatory properties of MBNL and CELF proteins...
  37. doi Absence of a differentiation defect in muscle satellite cells from DM2 patients
    Richard Pelletier
    Human Genetics Research Unit, Laval University, CHUQ, Pavillon CHUL, Ste Foy, Quebec, Canada, G1V 4G2
    Neurobiol Dis 36:181-90. 2009
    ..Finally, CUGBP1 steady-state protein levels were unchanged in DM2 cultured muscle cells and in DM2 muscle biopsies relative to ..
  38. pmc ETR-3 and CELF4 protein domains required for RNA binding and splicing activity in vivo
    Gopal Singh
    Department of Pathology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Nucleic Acids Res 32:1232-41. 2004
    ..These results identify specific regions of ETR-3 and CELF4 that are likely targets of protein-protein interactions required for splicing activation...
  39. ncbi Cloning and embryonic expression patterns of the chicken CELF family
    Kyle R Brimacombe
    Department of Cell Biology, Lerner Research Institute, Cleveland Clinic and Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, Ohio 44195, USA
    Dev Dyn 236:2216-24. 2007
    ..This suggests that different members of the CELF family play distinct functional roles during embryogenesis...
  40. pmc Expression of RNA CCUG repeats dysregulates translation and degradation of proteins in myotonic dystrophy 2 patients
    Elizabeth Salisbury
    Department of Molecular Physiology and Biophysics, Baylor College of Medicine, One Baylor Plaza, Houston, Texas 77030, USA
    Am J Pathol 175:748-62. 2009
    ..The second complex was found to contain CUG triplet repeat RNA-binding protein 1 (CUGBP1) and the translation initiation factor eIF2...
  41. doi Differential expression of the Brunol/CELF family genes during Xenopus laevis early development
    Jingyang Wu
    CAS Max Planck Junior Scientist Group, State Key Laboratory of Genetic Resources and Evolution, Kunming Institute of Zoology, Chinese Academy of Sciences, Kunming, China
    Int J Dev Biol 54:209-14. 2010
    ..Among them, only Brunol2 is maternally expressed and the zygotic expression of the other four Brunol genes starts at different ..
  42. pmc The neurofibromatosis type I pre-mRNA is a novel target of CELF protein-mediated splicing regulation
    Victoria A Barron
    Department of Genetics, School of Medicine, Case Western Reserve University, Cleveland, OH 44106, USA
    Nucleic Acids Res 38:253-64. 2010
    ..These studies, combined with our previous investigations demonstrating a role for Hu proteins and TIA-1/TIAR in controlling NF1 exon 23a inclusion, highlight the complex nature of regulation of this important alternative splicing event...
  43. pmc Systematic analysis of cis-elements in unstable mRNAs demonstrates that CUGBP1 is a key regulator of mRNA decay in muscle cells
    Jerome E Lee
    Program in Cell and Molecular Biology, Colorado State University, Fort Collins, Colorado, USA
    PLoS ONE 5:e11201. 2010
    ..Moreover, just as transcription factor activity varies between different cell types, the factors influencing mRNA decay are also cell-type specific...
  44. doi Clean STD-NMR spectrum for improved detection of ligand-protein interactions at low concentration of protein
    Youlin Xia
    Department of Biology and Biochemistry, University of Houston, Houston, TX 77004, USA
    Magn Reson Chem 48:918-24. 2010
    ..of sensitivity enhancement for a loose binding complex involving DNA oligonucleotide and an RNA-binding protein, CUGBP-1ab (25.2 kDa). The interesting binding characteristics of the complex dCTGTCT-CUGBP1ab were obtained...
  45. ncbi ETR-1, a homologue of a protein linked to myotonic dystrophy, is essential for muscle development in Caenorhabditis elegans
    C A Milne
    Medical Research Council, Laboratory of Molecular Biology, Hills Road, Cambridge, CB2 2QH, UK
    Curr Biol 9:1243-6. 1999
    ..elegans, perhaps by playing a role in post-transcriptional processing of some muscle component, and thus suggesting a possible conservation of gene function with human CUG-bp...
  46. pmc In vivo co-localisation of MBNL protein with DMPK expanded-repeat transcripts
    M Fardaei
    Institute of Genetics, University of Nottingham, Queen s Medical Centre, Nottingham NG7 2UH, UK
    Nucleic Acids Res 29:2766-71. 2001
    ..GFP-tagged MBNL does not appear as foci in non-DM1 cell lines. This work provides further support for the involvement of MBNL in DM1...
  47. ncbi Increased CUG triplet repeat-binding protein-1 predisposes to impaired adipogenesis with aging
    Iordanes Karagiannides
    Medicine and Biochemistry, Boston University, Boston, Massachusetts 02118, USA
    J Biol Chem 281:23025-33. 2006
    ..CUG triplet repeat-binding protein-1 (CUGBP1) binds to C/EBPbeta mRNA, increasing C/EBPbeta-LIP translation...
  48. ncbi A ferritin-responsive internal ribosome entry site regulates folate metabolism
    Collynn F Woeller
    Graduate Field of Biochemistry, Molecular and Cellular Biology, Cornell University, Ithaca, New York 14853, USA
    J Biol Chem 282:29927-35. 2007
    ..H ferritin was shown to interact with the mRNA-binding protein CUGBP1, a protein known to interact with the alpha and beta subunits of eukaryotic initiation factor eIF2...
  49. doi GU-rich RNA: expanding CUGBP1 function, broadening mRNA turnover
    Hyeon Ho Kim
    Laboratory of Cellular and Molecular Biology, National Institute on Aging Intramural Research Program, National Institutes of Health, Baltimore, MD 21224, USA
    Mol Cell 29:151-2. 2008
    In this issue of Molecular Cell, Vlasova et al. (2008) identify the GU-rich element (GRE) as a novel, widespread, degradation-promoting sequence through which the RNA-binding protein CUGBP1 elicits mRNA decay.
  50. pmc Pathogenic mechanisms of myotonic dystrophy
    Johanna E Lee
    Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
    Biochem Soc Trans 37:1281-6. 2009
    ..The RNA results in a toxic effect through two RNA-binding proteins: MBNL1 (muscleblind-like 1) and CUGBP1 (CUG-binding protein 1)...
  51. pmc Heart-specific overexpression of CUGBP1 reproduces functional and molecular abnormalities of myotonic dystrophy type 1
    Misha Koshelev
    Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
    Hum Mol Genet 19:1066-75. 2010
    ..effects include: sequestration of muscleblind-like 1 (MBNL1) protein and up-regulation of CUG binding protein 1 (CUGBP1). MBNL1 and CUGBP1 are RNA binding proteins that regulate alternative splicing transitions during development...
  52. doi Advanced oxidation protein products inhibit differentiation and activate inflammation in 3T3-L1 preadipocytes
    Qiu Gen Zhou
    Division of Nephrology, Nanfang Hospital, Southern Medical University, Guangzhou, China
    J Cell Physiol 225:42-51. 2010
    ..resulted in upregulated expression of C/EBP homologous protein (CHOP) and CUG-Triplet repeat-binding protein (CUGBP), two important inhibitors of preadipocytes differentiation...
  53. doi CELF and PTB proteins modulate the inclusion of the β-tropomyosin exon 6B during myogenic differentiation
    Alain Sureau
    Centre de Genetique Moleculaire, FRE 3144, CNRS, Gif sur Yvette, France, Universite Paris XI, Orsay, France
    Exp Cell Res 317:94-106. 2011
    ..Our results suggest that a change in the protein level of CUGBP1 and PTB proteins, associated with a distinct pattern of PTB during the transition from myoblasts to myotubes is ..
  54. pmc Muscle weakness in myotonic dystrophy associated with misregulated splicing and altered gating of Ca(V)1.1 calcium channel
    Zhen Zhi Tang
    Department of Neurology, University of Rochester Medical Center, Rochester, NY 14642, USA
    Hum Mol Genet 21:1312-24. 2012
    ..Two splicing factors previously implicated in DM1, MBNL1 and CUGBP1, participated in the regulation of E29 splicing. In muscle fibers of wild-type mice, the Ca(V)1...
  55. pmc The importance of CELF control: molecular and biological roles of the CUG-BP, Elav-like family of RNA-binding proteins
    Twishasri Dasgupta
    Department of Cell Biology, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, USA
    Wiley Interdiscip Rev RNA 3:104-21. 2012
    ..Dysregulation of CELF-mediated programs has been implicated in the pathogenesis of human diseases affecting the heart, skeletal muscles, and nervous system...
  56. doi Four parameters increase the sensitivity and specificity of the exon array analysis and disclose 25 novel aberrantly spliced exons in myotonic dystrophy
    Yoshihiro Yamashita
    Division of Neurogenetics, Center for Neurological Diseases and Cancer, Nagoya University Graduate School of Medicine, Nagoya, Japan
    J Hum Genet 57:368-74. 2012
    ..CTG repeats of DMPK sequestrate a splicing trans-factor MBNL1 and upregulate another splicing trans-factor CUGBP1. To identify a diverse array of aberrantly spliced genes, we performed the exon array analysis of DM1 muscles...
  57. pmc Bcl-x pre-mRNA splicing regulates brain injury after neonatal hypoxia-ischemia
    Qingli Xiao
    Hope Center for Neurological Disorders and Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Neurosci 32:13587-96. 2012
    ..We found that the splicing factor, CUG-binding-protein-1 (CUGBP1), bound to a consensus sequence close to the Bcl-x(L) 5' splice site, altering the Bcl-x(L)/Bcl-x(S) ratio and ..
  58. pmc Gene expression analyses implicate an alternative splicing program in regulating contractile gene expression and serum response factor activity in mice
    Twishasri Dasgupta
    Department of Cellular and Molecular Medicine, Lerner Research Institute, Cleveland Clinic, Cleveland, Ohio, USA
    PLoS ONE 8:e56590. 2013
    ..and downstream SRF targets are down-regulated in the hearts of MCKCUG-BP1 mice, which mildly over-express CELF1 in heart and skeletal muscle...
  59. pmc Transcriptional and translational regulation of C/EBPβ-HDAC1 protein complexes controls different levels of p53, SIRT1, and PGC1α proteins at the early and late stages of liver cancer
    Jingling Jin
    Huffington Center on Aging and Department of Pathology and Immunology, Baylor College of Medicine, Houston, Texas 77030, USA
    J Biol Chem 288:14451-62. 2013
    ..The RNA binding protein CUGBP1 is a key regulator of translation of CCAAT enhancer binding protein β and histone deacetylase 1 (HDAC1)...
  60. pmc Celf1 is required for formation of endoderm-derived organs in zebrafish
    Naoyuki Tahara
    Gene Regulation Research, Nara Institute Science and Technology, 8916 5 Takayama, Nara 630 0101, Japan
    Int J Mol Sci 14:18009-23. 2013
    We recently reported that an RNA binding protein called Cugbp Elav-like family member 1 (Celf1) regulates somite symmetry and left-right patterning in zebrafish...
  61. pmc Jnk2 deletion disrupts intestinal mucosal homeostasis and maturation by differentially modulating RNA-binding proteins HuR and CUGBP1
    Hee Kyoung Chung
    Cell Biology Group, Department of Surgery, University of Maryland School of Medicine, Baltimore, Maryland Baltimore Veterans Affairs Medical Center, Baltimore, Maryland
    Am J Physiol Cell Physiol 306:C1167-75. 2014
    ..in the level of the RNA-binding protein HuR and with a decrease in the abundance of CUG-binding protein 1 (CUGBP1)...
  62. pmc Development of an AP-FRET based analysis for characterizing RNA-protein interactions in myotonic dystrophy (DM1)
    Shagufta Rehman
    Department of Pathology, University of Virginia, Charlottesville, Virginia, United States of America
    PLoS ONE 9:e95957. 2014
    ..g. mbnl1, hnrnph, cugbp1)...
  63. doi Genetic variation at the CELF1 (CUGBP, elav-like family member 1 gene) locus is genome-wide associated with Alzheimer's disease and obesity
    Anke Hinney
    Department of Child and Adolescent Psychiatry, Psychotherapy, and Psychosomatics, Universitatsklinikum Essen, University of Duisburg Essen, Essen, Germany
    Am J Med Genet B Neuropsychiatr Genet 165:283-93. 2014
    ..1 × 10(-08)) at the locus CELF1 is also genome-wide significant for obesity (pBMI  = 7.35 × 10(-09) )...
  64. pmc Competition between RNA-binding proteins CELF1 and HuR modulates MYC translation and intestinal epithelium renewal
    Lan Liu
    Cell Biology Group, Department of Surgery, University of Maryland School of Medicine, Baltimore, MD 21201 Baltimore Veterans Affairs Medical Center, Baltimore, MD 21201
    Mol Biol Cell 26:1797-810. 2015
    ..The RNA-binding protein (RBP) ELAV-like family member 1 (CELF1), also referred to as CUG-binding protein 1 (CUGBP1), regulates the stability and translation of target mRNAs and is implicated in many aspects of cellular physiology...
  65. ncbi Exercise, skeletal muscle and inflammation: ARE-binding proteins as key regulators in inflammatory and adaptive networks
    Thomas Beiter
    Department of Sports Medicine, Medical Clinic, Eberhard Karls University Tübingen, Tübingen, Germany
    Exerc Immunol Rev 21:42-57. 2015
    ..The best-characterized ARE-binding proteins are HuR, CUGBP1, KSRP, AUF1, and the three ZFP36 proteins, especially TTP/ZFP36...
  66. pmc Antagonistic regulation of mRNA expression and splicing by CELF and MBNL proteins
    Eric T Wang
    Department of Biology, Massachusetts Institute of Technology, Cambridge, Massachusetts 02142, USA Koch Institute for Integrative Cancer Research, Massachusetts Institute of Technology, Cambridge, Massachusetts 02142, USA
    Genome Res 25:858-71. 2015
    RNA binding proteins of the conserved CUGBP1, Elav-like factor (CELF) family contribute to heart and skeletal muscle development and are implicated in myotonic dystrophy (DM)...
  67. pmc Destabilization of microRNAs in human cells by 3' deadenylation mediated by PARN and CUGBP1
    Takayuki Katoh
    Department of Chemistry and Biotechnology, Graduate School of Engineering, University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8656, Japan
    Nucleic Acids Res 43:7521-34. 2015
    ..We also demonstrate that CUG-binding protein 1 (CUGBP1) specifically interacts with miR-122 and other UG-rich miRNAs, and promotes their destabilization...
  68. pmc Overexpression of miR-214-3p in esophageal squamous cancer cells enhances sensitivity to cisplatin by targeting survivin directly and indirectly through CUG-BP1
    P Phatak
    Cell Biology Group, Department of Surgery, University of Maryland School of Medicine, Baltimore, MD, USA
    Oncogene 35:2087-97. 2016
    ..These findings suggest that miR-214-3p acts as a tumor suppressor and that its downregulation contributes to chemoresistance in esophageal cancer cells by targeting both survivin and CUG-BP1. ..
  69. pmc CUG-binding protein 1 regulates HSC activation and liver fibrogenesis
    Xingxin Wu
    State Key Laboratory of Pharmaceutical Biotechnology and Collaborative Innovation Center of Chemistry for Life Sciences, School of Life Sciences, Nanjing University, Nanjing 210023, China
    Nat Commun 7:13498. 2016
    ..Here we report that CUG-binding protein 1 (CUGBP1) expression is elevated in HSCs and positively correlates with liver fibrosis severity in human liver biopsies...
  70. pmc Overexpression of CUGBP1 in skeletal muscle from adult classic myotonic dystrophy type 1 but not from myotonic dystrophy type 2
    Rosanna Cardani
    Laboratory of Muscle Histopathology and Molecular Biology, IRCCS Policlinico San Donato, Milan, Italy
    PLoS ONE 8:e83777. 2013
    ..alternative splicing of specific pre-mRNAs by altering the functions of two RNA binding proteins, MBNL1 and CUGBP1. However, DM1 and DM2 show disease-specific features that make them clearly separate diseases suggesting that ..
  71. ncbi A trans-acting factor, isolated by the three-hybrid system, that influences alternative splicing of the amyloid precursor protein minigene
    A Poleev
    Max Planck Institute for Neurobiology, Munich, Germany
    Eur J Biochem 267:4002-10. 2000
    ..We conclude that clone AF169013 and its counterpart in human CUG-BP could be the trans-acting factors that interact with the splicing enhancer downstream of exon 8, and in this way influence alternative splicing of the APP minigene...
  72. ncbi Regulation of alternative splicing of alpha-actinin transcript by Bruno-like proteins
    Hitoshi Suzuki
    Graduate School of Biological Sciences, Nara Institute of Science and Technology, Ikoma 630 0101, Japan
    Genes Cells 7:133-41. 2002
    The Bruno-like or CELF proteins, such as mammalian CUGBP1 and Etr-3, Xenopus EDEN-BP, and Drosophila Bruno (Bru), are regulators of gene expression at the post-transcriptional level, and contain three RNA-recognition motifs (RRMs)...
  73. ncbi PABPN1 overexpression leads to upregulation of genes encoding nuclear proteins that are sequestered in oculopharyngeal muscular dystrophy nuclear inclusions
    Louis Philippe Corbeil-Girard
    Laboratoire de Neurogénétique, Centre de Recherche du CHUM, Universite de Montreal, Montreal, Quebec, Canada H2L 4M1
    Neurobiol Dis 18:551-67. 2005
    ..that all tested nuclear proteins encoded by eight upregulated genes colocalize with PABPN1 within the INIs: CUGBP1, SFRS3, FKBP1A, HMG2, HNRPA1, PRC1, S100P, and HSP70...
  74. pmc RNA-binding proteins hnRNP A2/B1 and CUGBP1 suppress fragile X CGG premutation repeat-induced neurodegeneration in a Drosophila model of FXTAS
    Oyinkan A Sofola
    Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Neuron 55:565-71. 2007
    ..Here, we show that overexpression of RNA-binding proteins hnRNP A2/B1 and CUGBP1 suppresses the phenotype of the CGG transgenic fly...
  75. ncbi The regulatory element in the 3'-untranslated region of human papillomavirus 16 inhibits expression by binding CUG-binding protein 1
    Rafal Goraczniak
    Department of Molecular Biology and Biochemistry, Rutgers University, Piscataway, New Jersey 08854, USA
    J Biol Chem 283:2286-96. 2008
    ..UV cross-linking, RNA affinity chromatography, and mass spectrometry that is bound by the CUG-binding protein 1 (CUGBP1)...
  76. pmc Systems genetics analysis of mouse chondrocyte differentiation
    Jaijam Suwanwela
    Department of Oral Biology, School of Dentistry, UCLA, Los Angeles, CA 90095, USA
    J Bone Miner Res 26:747-60. 2011
    ..Hspd1 and Cdkn1a, were known previously to function in chondrocyte development, whereas the other three, Bhlhb9, Cugbp1, and Spcs3, are novel genes...
  77. pmc Etiology of a genetically complex seizure disorder in Celf4 mutant mice
    J L Wagnon
    The Jackson Laboratory, Bar Harbor, ME 04609 1500, USA
    Genes Brain Behav 10:765-77. 2011
    Mice deficient for the gene encoding the RNA-binding protein CELF4 (CUGBP, ELAV-like family member 4) have a complex seizure phenotype that includes both convulsive and non-convulsive seizures, depending upon gene dosage and strain ..
  78. doi Viral delivery of miR-196a ameliorates the SBMA phenotype via the silencing of CELF2
    Yu Miyazaki
    Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan
    Nat Med 18:1136-41. 2012
    ..We found that miR-196a enhanced the decay of the AR mRNA by silencing CUGBP, Elav-like family member 2 (CELF2). CELF2 directly acted on AR mRNA and enhanced the stability of AR mRNA...
  79. pmc Alternative splicing of the neurofibromatosis type 1 pre-mRNA is regulated by the muscleblind-like proteins and the CUG-BP and ELAV-like factors
    Victoria A Fleming
    Department of Genetics, Case Western Reserve University, Cleveland, OH, 44106, USA
    BMC Mol Biol 13:35. 2012
    ..Currently only a limited number of common targets of MBNL and CELF are known that are antagonistically regulated by these two groups of proteins...
  80. doi CUG-binding protein 1 (CUGBP1) expression and prognosis of non-small cell lung cancer
    W Jiao
    Department of Thoracic Surgery, the Affiliated Hospital of Medical College, Qingdao University, 16 Jiangsu Road, Qingdao, 266003, People s Republic of China
    Clin Transl Oncol 15:789-95. 2013
    Non-small cell lung cancer (NSCLC) is the leading cause of cancer mortality worldwide. As CUGBP1 may also play a great role in tumor genesis and deterioration, the purpose of this study was to detect the expression of CUGBP1 mRNA and ..
  81. doi A defective Krab-domain zinc-finger transcription factor contributes to altered myogenesis in myotonic dystrophy type 1
    Morgane Gauthier
    INSERM UMR 861, I STEM AFM, Evry Cedex 91030, France
    Hum Mol Genet 22:5188-98. 2013
    ..this defect in expression, which derives from a loss of RNA stability, is controlled by the RNA-binding protein, CUGBP1, and is associated with impaired myogenesis-a functional defect reminiscent of that observed in DM1...
  82. pmc Antagonistic regulation of alpha-actinin alternative splicing by CELF proteins and polypyrimidine tract binding protein
    Natalia Gromak
    Department of Biochemistry, University of Cambridge, Cambridge CB2 1GA, UK
    RNA 9:443-56. 2003
    ....
  83. pmc The circadian RNA-binding protein CHLAMY 1 represents a novel type heteromer of RNA recognition motif and lysine homology domain-containing subunits
    Bin Zhao
    Institut fur Allgemeine Botanik, Friedrich Schiller Universitat Jena, Am Planetarium 1, 07743 Jena, Germany
    Eukaryot Cell 3:815-25. 2004
    ..Notably, the C3 subunit shows significant homology to the rat CUG-binding protein 2. Anti-C3 antibodies can recognize the rat homologue, which can also be found in a protein complex in this vertebrate...
  84. pmc The role of CUGBP1 in age-dependent changes of liver functions
    Karlie Jones
    Department of Molecular Physiology and Biophysics, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Ageing Res Rev 11:442-9. 2012
    ..This review is focused on the role of a conserved, multi-functional RNA-binding protein, CUGBP1, in the development of aging phenotype in the liver...
  85. doi Complex evolutionary relationships among four classes of modular RNA-binding splicing regulators in eukaryotes: the hnRNP, SR, ELAV-like and CELF proteins
    Yue Hang Tang
    School of Chemistry and Molecular Biosciences, University of Queensland, Brisbane, QLD, Australia
    J Mol Evol 75:214-28. 2012
    ....
  86. pmc CUG-BP, Elav-like family (CELF)-mediated alternative splicing regulation in the brain during health and disease
    Andrea N Ladd
    Department of Cellular and Molecular Medicine, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, USA Electronic address
    Mol Cell Neurosci 56:456-64. 2013
    ..This article is part of a Special Issue entitled "RNA and splicing regulation in neurodegeneration." ..
  87. pmc Diversity and conservation of CELF1 and CELF2 RNA and protein expression patterns during embryonic development
    Y Blech-Hermoni
    Department of Cellular and Molecular Medicine, Lerner Research Institute, Cleveland Clinic, Cleveland, Ohio, USA
    Dev Dyn 242:767-77. 2013
    CUG-BP, Elav-like family member 1 (CELF1) and CELF2 are RNA-binding proteins that regulate several stages of RNA processing, and are broadly expressed in developing and adult tissues...
  88. pmc Smaug/SAMD4A restores translational activity of CUGBP1 and suppresses CUG-induced myopathy
    Maria de Haro
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, United States of America
    PLoS Genet 9:e1003445. 2013
    ..Using human myoblasts, we show physical interactions between human Smaug (SMAUG1/SMAD4A) and CUGBP1. Increased levels of SMAUG1 correct the abnormally high nuclear accumulation of CUGBP1 in myoblasts from DM1 ..
  89. pmc Molecular mechanisms of muscle atrophy in myotonic dystrophies
    Lubov Timchenko
    Department of Molecular Physiology and Biophysics, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Int J Biochem Cell Biol 45:2280-7. 2013
    ..that alter RNA metabolism in patients' tissues by targeting RNA-binding proteins such as CUG-binding protein 1 (CUGBP1) and Muscle blind-like protein 1 (MBNL1)...
  90. ncbi Cardiac elav-type RNA-binding protein (ETR-3) binds to RNA CUG repeats expanded in myotonic dystrophy
    X Lu
    Departments of Medicine, Section of Cardiology and Pathology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Hum Mol Genet 8:53-60. 1999
    ..Our results suggest the existence of a family of proteins that bind to CUG repeats and might be affected in DM by expansion of CUG repeats...
  91. ncbi A family of human RNA-binding proteins related to the Drosophila Bruno translational regulator
    P J Good
    Department of Biochemistry and Molecular Biology and Feist Weiller Cancer Center, Louisiana State University, Health Sciences Center, Shreveport, Lousiana 71130, USA
    J Biol Chem 275:28583-92. 2000
    ..The human BRUNOL2 and BRUNOL3 are 92% identical in the RNA-binding domains, although the BRUNOL2 gene is expressed ubiquitously ..
  92. ncbi Coexpression of the CUG-binding protein reduces DM protein kinase expression in COS cells
    N Takahashi
    Department of Life Sciences, Graduate School of Arts and Sciences, The University of Tokyo, Komaba, Meguro ku, Tokyo 153 8902, Japan
    J Biochem 130:581-7. 2001
    ..On the other hand, cotransfection of CUG-BP significantly affected the expression of both the wild type and mutant DMPKs at the protein level. This reduction was remarkable when the mutant DMPK construct was used...
  93. ncbi c-Jun ARE targets mRNA deadenylation by an EDEN-BP (embryo deadenylation element-binding protein)-dependent pathway
    Luc Paillard
    CNRS UMR 6061, Universite de Rennes 1, Faculte de Medecine, 2 avenue Léon Bernard, 35043 Rennes Cedex, France
    J Biol Chem 277:3232-5. 2002
    ..Together, these results identify CUG-BP as a plausible deadenylation factor responsible for the post-transcriptional control of c-Jun proto-oncogene mRNA in mammalian cells...
  94. ncbi Loss of the muscle-specific chloride channel in type 1 myotonic dystrophy due to misregulated alternative splicing
    Nicolas Charlet-B
    Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA
    Mol Cell 10:45-53. 2002
    ..We propose that disruption of alternative splicing regulation causes a predominant pathological feature of DM1...
  95. ncbi A functional deadenylation assay identifies human CUG-BP as a deadenylation factor
    Luc Paillard
    CNRS UMR 6061, Faculte de Medecine, Universite Rennes 1, 2, avenue Léon Bernard, CS 34317, 35043 cedex, Rennes, France
    Biol Cell 95:107-13. 2003
    ..The results presented here identify CUG-BP-dependent deadenylation as a potential cytoplasmic target for this trans-dominant effect...
  96. ncbi Overexpression of CUG triplet repeat-binding protein, CUGBP1, in mice inhibits myogenesis
    Nikolai A Timchenko
    Department of Pathology and Huffington Center on Aging, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    J Biol Chem 279:13129-39. 2004
    ..of RNA CUG repeats in myotonic dystrophy type 1 (DM1) patients leads to the induction of a CUG-binding protein, CUGBP1, which increases translation of several proteins that are required for myogenesis...
  97. ncbi Muscleblind protein, MBNL1/EXP, binds specifically to CHHG repeats
    Yoshihiro Kino
    Department of Life Sciences, Graduate School of Arts and Sciences, University of Tokyo, Komaba, Meguro ku, Tokyo, Japan
    Hum Mol Genet 13:495-507. 2004
    ..It was also found that MBNL1 can bind to repetitive motifs in ZNF9, which contain a minimal length of CCUG repeats with non-CCUG insertions...
  98. pmc The Drosophila Bruno paralogue Bru-3 specifically binds the EDEN translational repression element
    Jérôme Delaunay
    Institut de Genetique Humaine, UPR 1142 CNRS, 141 rue de la Cardonille, 34396 Montpellier Cedex 5, France
    Nucleic Acids Res 32:3070-82. 2004
    ..The phylogenetic analyses and the experimental data based on its specific binding to the EDEN element support the conclusion that Bru-3 is an EDEN-BP/CUG-BP orthologue...
  99. ncbi RNA CUG-binding protein 1 increases translation of 20-kDa isoform of CCAAT/enhancer-binding protein beta by interacting with the alpha and beta subunits of eukaryotic initiation translation factor 2
    Nikolai A Timchenko
    Department of Pathology and Huffington Center on Aging, Baylor College of Medicine, Houston, Texas 77030, USA
    J Biol Chem 280:20549-57. 2005
    ..Two RNA-binding proteins, CUGBP1 and calreticulin, have been implicated in the translational regulation of C/EBPbeta...
  100. ncbi Dynamic balance between activation and repression regulates pre-mRNA alternative splicing during heart development
    Andrea N Ladd
    Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA
    Dev Dyn 233:783-93. 2005
    ..ETR-3 and CUG-BP proteins are also down-regulated in other tissues during development, suggesting that CELF proteins play a broad role in developmental splicing regulation...
  101. pmc Identification of CELF splicing activation and repression domains in vivo
    Jin Han
    Department of Pathology, Baylor College of Medicine Houston, TX 77030, USA
    Nucleic Acids Res 33:2769-80. 2005
    ..This analysis was performed in parallel on two CELF proteins, ETR-3 (CUG-BP2, NAPOR, BRUNOL3) and CELF4 (BRUNOL4)...

Research Grants26

  1. Molecular mechanisms of Myotonic Dystrophy 1
    Lubov T Timchenko; Fiscal Year: 2012
    ..CUG repeats misregulate RNA-binding proteins, including CUGBP1. CUGBP1 is a multifunctional protein which regulates translation, RNA stability and splicing...
  2. Thomas A Cooper; Fiscal Year: 2016
    ..kinase C (PKC) and induces PKC-dependent phosphorylation of a second RNA binding protein, CUG-binding protein 1 (CUGBP1), resulting in its stabilization and up-regulation...
  3. Regulation of Cell Growth by RNA Binding Proteins
    Nikolai A Timchenko; Fiscal Year: 2012
    ..My laboratory investigates biological activities of RNA CUG triplet repeat binding protein, CUGBP1, and the role of this protein in liver proliferation and in liver tumors...
  4. Genetic Regulation of Complex Neurological Disease
    Wayne N Frankel; Fiscal Year: 2012
    ..was identified in mice, in the gene encoding Brunol4, a brain-specific, hippocampus-enriched member of the Bruno/CUGBP/CELF family of RNA binding proteins...
  5. Genetic Regulation of Complex Neurological Diseases
    Wayne N Frankel; Fiscal Year: 2013
    ..hypomorphic and null genotypes in Celf4 (formerly known as Brunol4), encoding a brain-specific member of the BRUNO/CUGBP/CELF family of RNA binding proteins...
  6. Andrew Berglund; Fiscal Year: 2014
    ..act as toxic RNAs to sequester MBNL1, a RNA binding protein, and also indirectly increase the protein levels of CUGBP1, another RNA binding protein, which disrupts the normal cellular function of these proteins...
  7. The toxicity of the RNA CGG repeats in FXTAS
    Lubov T Timchenko; Fiscal Year: 2013
    ..We also observed that rCGG repeats increase the levels of un-phosphorylated at S302 form of RNA-binding protein, CUGBP1, named CUGBP1REP...
  8. Maha Abdellatif; Fiscal Year: 2014
    ..2) AKT phosphorylates the RNA-binding protein CUGBP1, which binds the loop region of primary miR-21 and enhances its processing, thus, increasing mature miR-21 levels...
  9. Eric T Wang; Fiscal Year: 2016
    ..CUG repeat expression also leads to hyper-phosphorylation of the CUGBP/ ELAV-like factors (CELFs), resulting in CELF protein elevation...
  10. MAURICE SCOTT SWANSON; Fiscal Year: 2014
    ..because they alter the activities of two families of alternative splicing factors, the muscleblind-like (MBNL) and CUGBP/ETR-3-like factor (CELF) proteins...
  11. DAVID LOREN NELSON; Fiscal Year: 2016
    ..Both Pur 1 and the heterodimer complex, hnRNP A2/CUGBP1, could modulate the rCGG-mediated toxicity in Drosophila model, supporting RNA-mediated sequestration model of ..
  12. Artificial site-specific RNA endonuclease as new Myotonic Dystrophy therapeutics
    Zefeng Wang; Fiscal Year: 2012
    ..of ASRE in DM1 cells can restore the normal expression level and localization pattern of CUG-binding protein 1 (CUGBP1) and muscle blind-like 1 (MBNL1)...
  13. MOLECULAR MECHANISMS OF MYOTONIC DYSTROPHY
    JOHN A BERGLUND; Fiscal Year: 2010
    ..Our goal is to understand the molecular mechanisms that cause DM. This understanding will help lead to the development of therapies to help the many people (1 in 8000) that have this most common form of adult onset muscular dystrophy. ..
  14. Effect of Aging on Preadipocyte Differentiation
    James L Kirkland; Fiscal Year: 2012
    ..animals, increased TNF1 release by preadipocytes with aging, and stress-responsive, anti-adipogenic regulators (CUGBP, CHOP) all impede adipogenesis in old rats...
  15. RNA mediated Neurotoxicity in Huntington's Disease-Like 2 and Huntington Disease
    DOBRILA DODA RUDNICKI; Fiscal Year: 2013
    ..We also predict that other CUG repeat binding proteins, including CUGBP1, may influence disease pathogenesis. We will test this hypothesis in a series of experiments in 3 specific aims...
  16. Carol J Wilusz; Fiscal Year: 2014
    ..In addition, the mutant RNA induces aberrant expression of another RNA-binding protein, CUGBP1 through an unknown mechanism. This in turn impacts processing and decay of additional mRNAs...
  17. Jian Ying Wang; Fiscal Year: 2015
    ..proposed in this competitive renewal application are to test the HYPOTHESIS that the RNA-binding protein CUGBP1 plays an important role in the regulation of intestinal mucosal growth by altering expression of its target mRNAs ..
  18. Deep Sequencing Analysis of mRNA Isoform Expression Changes in Myotonic Dystrophy
    David Housman; Fiscal Year: 2010
    ..alternative splicing factor Muscleblind (MBNL1) and hyperactivation of the splicing factor CUG Binding Protein 1 (CUGBP1). Misregulation of splicing by these factors is central in the disease...
  19. ANDREA NICOLE LADD; Fiscal Year: 2014
    ..Furthermore, these studies will shed light on how disruption of normal alternative splicing contributes to disease states resulting from developmental perturbation ..
  20. Sui Huang; Fiscal Year: 2015
    ..The complex contains PTB, CUGBP, RMRP, and other unknown factors, and is large, pelleting at the bottom of a 5-40% glycerol gradient...
  21. C/EBPbeta Isoform Function & Regulation by Tyrosine Kinases
    Cynthia Zahnow; Fiscal Year: 2009
    ..Furthermore, EGFR-mediated-p44/42 MAPK signaling phosphorylates and activates the RNA binding protein, CUGBP1, which then binds to C/EBPB mRNA to regulate translation of the LIP isoform...
  22. Investigating mechanisms of skeletal muscle degeneration in Myotonic Dystrophy
    James P Orengo; Fiscal Year: 2010
    ..In Specific Aim 3 a splicing factor, CUGBP1, which regulates developmental alternative splicing patterns in post-natal development, will be investigated...
  23. ABERRANT RNA PROCESSING IN HUMAN DEVELOPMENT
    Adil Shamoo; Fiscal Year: 2002
    ..abstract_text> ..
  24. MOLECULAR MECHANISMS WHEREBY CTG EXPANSION RESULTS IN DM
    Sita Reddy; Fiscal Year: 2004
    ....
  25. C/EBP alpha in Aging Liver
    Nikolai Timchenko; Fiscal Year: 2009
    ..Based on data obtained in this project, we are planning to start developing a strategy to correct liver proliferation in elderly. ..
  26. The Role of RNA-Binding Proteins in Myogenesis
    Lubov Timchenko; Fiscal Year: 2007
    ..One of the members of this family, CUGBP1, is highly expressed in skeletal muscle and is implicated in the delay of skeletal muscle differentiation in ..