CELF1

Summary

Gene Symbol: CELF1
Description: CUGBP, Elav-like family member 1
Alias: BRUNOL2, CUG-BP, CUGBP, CUGBP1, EDEN-BP, NAB50, NAPOR, hNab50, 50 kDa nuclear polyadenylated RNA-binding protein, CELF-1, CUG RNA-binding protein, CUG triplet repeat RNA-binding protein 1, CUG triplet repeat, RNA binding protein 1, CUG triplet repeat, RNA-binding protein 1, CUG-BP- and ETR-3-like factor 1, CUG-BP1, CUGBP Elav-like family member 1, 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. pmc Inducible activation of CEBPB, a gene negatively regulated by BCR/ABL, inhibits proliferation and promotes differentiation of BCR/ABL-expressing cells
    Clara Guerzoni
    Department of Microbiology and Immunology, Kimmel Cancer Center, Thomas Jefferson Medical College, 233 South and 10th Street, Philadelphia, PA 19107, USA
    Blood 107:4080-9. 2006
  3. 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
  4. 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
  5. 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
  6. 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
  7. 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
  8. 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
  9. 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
  10. 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

Research Grants

  1. Myotonic Dystrophy Type 2
    Lubov Timchenko; Fiscal Year: 2009
  2. Targeting a Toxic RNA with Small Molecules
    JOHN A BERGLUND; Fiscal Year: 2010
  3. The Role of RNA-Binding Proteins in Myogenesis
    Lubov Timchenko; Fiscal Year: 2007
  4. RNA mediated Neurotoxicity in Huntington's Disease-Like 2 and Huntington Disease
    DOBRILA DODA RUDNICKI; Fiscal Year: 2010
  5. Novel Regulators of T Cell mRNA Decay
    Paul Bohjanen; Fiscal Year: 2009
  6. MOLECULAR PATHOGENESIS OF MYOTONIC DYSTROPHY
    THOMAS ALEXANDER COOPER; Fiscal Year: 2010
  7. MOLECULAR MECHANISMS OF MYOTONIC DYSTROPHY
    Lubov Timchenko; Fiscal Year: 2009
  8. MOLECULAR MECHANISMS OF MYOTONIC DYSTROPHY
    Lubov T Timchenko; Fiscal Year: 2010
  9. Novel Regulators of T Cell mRNA Decay
    Paul R Bohjanen; Fiscal Year: 2010
  10. Novel Regulators of T Cell mRNA Decay
    Paul R Bohjanen; Fiscal Year: 2010

Scientific Experts

  • L Paillard
  • Yuhuan Zheng
  • Yue Hang Tang
  • Lubov Timchenko
  • Takaaki Matsui
  • ANDREA NICOLE LADD
  • Jaijam Suwanwela
  • Jean Marc Gallo
  • Ken Fujimura
  • Youlin Xia
  • Hsiao Huei Chen
  • Richard Pelletier
  • Annalisa Botta
  • P J Good
  • Nikolai Timchenko
  • Thomas Cooper
  • James Kirkland
  • Cynthia Zahnow
  • JOHN BERGLUND
  • Thomas A Cooper
  • JOHN A BERGLUND
  • DAVID LOREN NELSON
  • Paul R Bohjanen
  • Adil Shamoo
  • THOMAS ALEXANDER COOPER
  • James P Orengo
  • Guo Li Wang
  • Elizabeth Salisbury
  • James L Kirkland
  • Yoshihiro Kino
  • MAURICE SCOTT SWANSON
  • DOBRILA RUDNICKI
  • DOBRILA DODA RUDNICKI
  • Paul Bohjanen
  • JAMES ORENGO
  • AMANDA JOY WARD
  • Steven C Zimmerman
  • Benedikt Schoser
  • Maria de Haro
  • Auinash Kalsotra
  • Guey Shin Wang
  • Victoria A Barron
  • Noboru Sasagawa
  • Shoichi Ishiura
  • Bernd Rattenbacher
  • Amanda J Ward
  • Claudia Huichalaf
  • Karlie Jones
  • Hua Lou
  • Daniel Beisang
  • Jerome E Lee
  • Christiane Schneider-Gold
  • Juan Botas
  • Keiko Sakai
  • Jennifer T Fox
  • Jeffrey Wilusz
  • John C Castle
  • Carol J Wilusz
  • Yuriko Nezu
  • Kyle R Brimacombe
  • Daisuke Mori
  • Sharan Paul
  • Y Blech-Hermoni
  • W Jiao
  • Warunee Dansithong
  • Rosanna Cardani
  • Morgane Gauthier
  • Douglas J Turner
  • Victoria A Fleming
  • Jin Han
  • Joëlle Marie
  • Jaladanki N Rao
  • Yu Miyazaki
  • Lan Xiao
  • Yu Hong Cui
  • Elizabeth T Chang
  • Jian Ying Wang
  • J L Wagnon
  • C M Dhaenens
  • Alain Sureau
  • Dara S Berger
  • Cristina Gareau
  • Luciano H Apponi
  • Ioannis Grammatikakis
  • Callie Pollock
  • Marie Cibois
  • Gopal Singh
  • Qiu Gen Zhou
  • Jingyang Wu
  • Misha Koshelev

Detail Information

Publications123 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. pmc Inducible activation of CEBPB, a gene negatively regulated by BCR/ABL, inhibits proliferation and promotes differentiation of BCR/ABL-expressing cells
    Clara Guerzoni
    Department of Microbiology and Immunology, Kimmel Cancer Center, Thomas Jefferson Medical College, 233 South and 10th Street, Philadelphia, PA 19107, USA
    Blood 107:4080-9. 2006
    ..by imatinib (STI571) via a mechanism that appears to depend on expression of the CUG-repeat RNA-binding protein CUGBP1 and the integrity of the CUG-rich intercistronic region of c/ebpbeta mRNA...
  3. 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)...
  4. 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...
  5. 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 ..
  6. 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 ..
  7. 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...
  8. 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...
  9. 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)...
  10. 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...
  11. 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...
  12. 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...
  13. 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 ..
  14. 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...
  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 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...
  17. 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...
  18. 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...
  19. 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...
  20. 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 ..
  21. 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...
  22. 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 ..
  23. 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...
  24. 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 ..
  25. 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)...
  26. 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)...
  27. 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 ..
  28. 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 ..
  29. 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...
  30. 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...
  31. 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%...
  32. 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...
  33. 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 ..
  34. 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...
  35. 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 ..
  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 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...
  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. 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 ..
  41. 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...
  42. 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...
  43. 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...
  44. 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...
  45. 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...
  46. 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...
  47. 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 ..
  48. 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...
  49. 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)...
  50. 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...
  51. 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 ..
  52. 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...
  53. 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 ..
  54. 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...
  55. 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...
  56. 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...
  57. 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...
  58. 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)...
  59. 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
    ....
  60. 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...
  61. 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...
  62. doi 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...
  63. 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)...
  64. 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 ..
  65. doi 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...
  66. 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." ..
  67. 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
    ....
  68. pmc The role of CELF proteins in neurological disorders
    Jean Marc Gallo
    MRC Centre for Neurodegeneration Research, King s College London, Institute of Psychiatry, Department of Clinical Neuroscience, De Crespigny Park, London UK
    RNA Biol 7:474-9. 2010
    ..The first member of the family, CELF1/CUG-BP1, was identified through its role in myotonic dystrophy, type 1...
  69. pmc BrunoL1 regulates endoderm proliferation through translational enhancement of cyclin A2 mRNA
    Lori Dawn Horb
    Laboratory of Molecular Organogenesis, Institut de Recherches Cliniques de Montreal, Montreal, QC, Canada
    Dev Biol 345:156-69. 2010
    ..of this functional information is derived from studies on mainly two members, Drosophila Bruno and vertebrate BrunoL2 (CUGBP1)...
  70. 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...
  71. pmc Myotonic dystrophies 1 and 2: complex diseases with complex mechanisms
    Benedikt Schoser
    Department of Neurology, Friedrich Baur Institute, Ludwig Maximilians University, Ziemssenstr 1a, Munich, Germany
    Curr Genomics 11:77-90. 2010
    ..The expanded CUGn and CCUGn RNAs mainly target two RNA binding proteins, MBNL1 and CUGBP1, elevating levels of CUGBP1 and reducing levels of MBNL1...
  72. pmc HTS-Compatible Patient-Derived Cell-Based Assay to Identify Small Molecule Modulators of Aberrant Splicing in Myotonic Dystrophy Type 1
    Debra A O'Leary
    Genomics Institute of the Novartis Research Foundation, 10675 John Jay Hopkins Drive, San Diego, CA 92121, USA
    Curr Chem Genomics 4:9-18. 2010
    ..genes through the dysregulation of RNA-binding proteins including Muscleblind 1 (MBNL1) and CUG binding protein 1 (CUGBP1)...
  73. 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 ..
  74. doi HER2 silences tumor suppression in breast cancer cells by switching expression of C/EBPß isoforms
    Anna Arnal-Estapé
    Oncology Programme, Institute for Research in Biomedicine, Barcelona, Spain
    Cancer Res 70:9927-36. 2010
    ..HER2 signaling activates the translational regulatory factor CUGBP1, which favors the production of the transcriptionally inhibitory isoform LIP over that of the active isoform LAP...
  75. pmc Identification of MBNL1 and MBNL3 domains required for splicing activation and repression
    Ioannis Grammatikakis
    Department of Pathology and Immunology, Baylor College of Medicine, Houston, TX 77030, USA
    Nucleic Acids Res 39:2769-80. 2011
    ..These results have identified protein domains required for splicing repression and activation and provide insight into the mechanism of splicing regulation by MBNL proteins...
  76. pmc Expression of a dominant negative CELF protein in vivo leads to altered muscle organization, fiber size, and subtype
    Dara S Berger
    Department of Cell Biology, Lerner Research Institute, Cleveland Clinic, Cleveland, Ohio, United States of America
    PLoS ONE 6:e19274. 2011
    ....
  77. pmc Alternative splicing of the neurofibromatosis type I pre-mRNA
    Victoria A Barron
    Department of Genetics, School of Medicine, Case Western Reserve University, 10900 Euclid Avenue, Cleveland, OH 44106, USA
    Biosci Rep 32:131-8. 2012
    ..We suggest that the regulation of alternative splicing may act as a modifier to contribute to the variable expression in NF1 patients...
  78. pmc miR-503 represses CUG-binding protein 1 translation by recruiting CUGBP1 mRNA to processing bodies
    Yu Hong Cui
    Department of Surgery, University of Maryland School of Medicine, Baltimore, MD 21201, USA
    Mol Biol Cell 23:151-62. 2012
    ..The RBP CUG-binding protein 1 (CUGBP1) destabilizes and represses the translation of several target mRNAs, but the exact mechanism that regulates CUGBP1 ..
  79. 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...
  80. pmc RNA-binding proteins and gene regulation in myogenesis
    Luciano H Apponi
    Department of Pharmacology, Emory University, Atlanta, GA 30322, USA
    Trends Pharmacol Sci 32:652-8. 2011
    ..review is to highlight the roles of the best-characterized RNA-binding proteins in muscle, including HuR, KSRP, CUGBP1, PABPN1, Lin-28 and TTP...
  81. pmc CUG-binding protein represses translation of p27Kip1 mRNA through its internal ribosomal entry site
    Yuhuan Zheng
    Cancer Biology Research Center, Sanford Research USD, Sioux Falls, and Division of Basic Biomedical Sciences, Sanford School of Medicine of the University of South Dakota, Vermillion, SD, USA
    RNA Biol 8:365-71. 2011
    ..In this study, the RNA-associated protein CUGBP1 was shown to interact with the human p27 5'-untranslated region...
  82. doi Mis-splicing of Tau exon 10 in myotonic dystrophy type 1 is reproduced by overexpression of CELF2 but not by MBNL1 silencing
    C M Dhaenens
    INSERM, U837 1, Alzheimer and Tauopathies, Place de Verdun, F 59045 Lille, France
    Biochim Biophys Acta 1812:732-42. 2011
    ..These are probably the consequence of a loss of muscleblind-like 1 (MBNL1) function or gain of CUGBP1 and ETR3-like factor 1 (CELF1) splicing function...
  83. pmc Alternative splicing dysregulation secondary to skeletal muscle regeneration
    James P Orengo
    Department of Pathology and Immunology and, Baylor College of Medicine Houston, TX 77030, USA
    Ann Neurol 69:681-90. 2011
    ..of muscleblindlike 1 (MBNL1) protein within nuclear RNA foci and increased CUGBP, ELAV-like family member 1 (CELF1) protein levels...
  84. pmc Characterization of MRP RNA-protein interactions within the perinucleolar compartment
    Callie Pollock
    Department of Cell and Molecular Biology, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA
    Mol Biol Cell 22:858-67. 2011
    ..mitochondrial RNA-processing (MRP) RNA, pyrimidine tract-binding protein (PTB), and CUG-binding protein (CUGBP) interact in vivo, as demonstrated by coimmunoprecipitation and RNA pull-down experiments...
  85. 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...
  86. pmc MicroRNAs coordinate an alternative splicing network during mouse postnatal heart development
    Auinash Kalsotra
    Department of Pathology and Immunology, Baylor College of Medicine, Houston, Texas 77030, USA
    Genes Dev 24:653-8. 2010
    ..We demonstrate a direct role for miR-23a/b in the dramatic postnatal down-regulation of CUGBP and ETR-3-like factor (CELF) proteins that regulate nearly half of developmentally regulated splicing transitions ..
  87. doi Chromosome wide analysis of CUGBP1 binding sites identifies the tetraspanin CD9 mRNA as a target for CUGBP1-mediated down-regulation
    Olivier Le Tonquèze
    CNRS, UMR 6061 Institut de Génétique et Développement de Rennes, Expression Génétique et Développement, Universite de Rennes I, IFR 140 GFAS, 2 Avenue du Professeur Leon Bernard, CS 34317, 35043 Rennes Cedex, France
    Biochem Biophys Res Commun 394:884-9. 2010
    b>CUGBP1 is an RNA-binding protein controlling alternative splicing, mRNA translation and stability. In this work we used a motif scoring approach to identify putative CUGBP1 binding sites for genes located on the human chromosome 12...
  88. pmc HDAC1 cooperates with C/EBPalpha in the inhibition of liver proliferation in old mice
    Guo Li Wang
    Huffington Center on Aging and Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA
    J Biol Chem 283:26169-78. 2008
    ..The elevation of HDAC1 in liver is mediated by the RNA-binding protein CUGBP1. We found that the age-associated CUGBP1-eIF2 complex binds to the 5' region of HDAC1 mRNA and increases ..
  89. 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...
  90. 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...
  91. pmc Expanded CTG repeats within the DMPK 3' UTR causes severe skeletal muscle wasting in an inducible mouse model for myotonic dystrophy
    James P Orengo
    Department of Pathology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Proc Natl Acad Sci U S A 105:2646-51. 2008
    ..regulated alternative splicing events, myotonia, characteristic histological abnormalities, and increased CUGBP1 protein levels...
  92. 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)...
  93. ncbi The Muscleblind family of proteins: an emerging class of regulators of developmentally programmed alternative splicing
    Maya Pascual
    Department of Genetics, University of Valencia, Doctor Moliner, 50, 46100 Burjasot, Valencia, Spain
    Differentiation 74:65-80. 2006
    ..Defining the mechanism of the activity of Muscleblind proteins, their splicing partners, and the functional relevance of its several protein isoforms are just a few examples...
  94. ncbi MBNL1 and CUGBP1 modify expanded CUG-induced toxicity in a Drosophila model of myotonic dystrophy type 1
    Maria de Haro
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA
    Hum Mol Genet 15:2138-45. 2006
    ..We also report that altering the levels of two RNA-BP known to be involved in DM1 pathogenesis, MBNL1 and CUGBP1, modify the (iCUG)480 degenerative phenotypes...
  95. ncbi Age-specific CUGBP1-eIF2 complex increases translation of CCAAT/enhancer-binding protein beta in old liver
    Lubov T Timchenko
    Huffington Center on Aging and Department of Pathology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    J Biol Chem 281:32806-19. 2006
    The RNA-binding protein CUGBP1 regulates translation of proteins in a variety of biological processes...
  96. 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...
  97. pmc Elevation of RNA-binding protein CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophy
    Guey Shin Wang
    Department of Pathology, Baylor College of Medicine, Houston, Texas, USA
    J Clin Invest 117:2802-11. 2007
    ..Combined in situ hybridization and immunofluorescent staining for CUGBP1 and CUGBP2, the 2 CUGBP1 and ETR-3 like factor (CELF) proteins expressed in heart, demonstrated elevated protein ..
  98. pmc Interaction of muscleblind, CUG-BP1 and hnRNP H proteins in DM1-associated aberrant IR splicing
    Sharan Paul
    Institute for Genetic Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033, USA
    EMBO J 25:4271-83. 2006
    ..These data demonstrate that coordinated physical and functional interactions between hnRNP H, CUG-BP1 and MBNL1 dictate IR splicing in normal and DM1 myoblasts...
  99. ncbi Gene expression analysis in myotonic dystrophy: indications for a common molecular pathogenic pathway in DM1 and DM2
    Annalisa Botta
    Department of Biopathology, Tor Vergata University of Rome, Rome, Italy
    Gene Expr 13:339-51. 2007
    ..No essential differences were observed in the muscle blind-like gene (MBNL1) and CUG binding protein 1 (CUGBP1) transcript levels as well as in the splicing pattern of the myotubularin-related 1 (MTMR1) gene...
  100. pmc Inactivation of CUG-BP1/CELF1 causes growth, viability, and spermatogenesis defects in mice
    Chantal Kress
    URA 2578 CNRS Institut Pasteur, 25 rue du Docteur Roux, 75724 Paris Cedex 15, France
    Mol Cell Biol 27:1146-57. 2007
    ..To elucidate its role in mammalian development, we produced mice in which the Cugbp1 gene was inactivated by homologous recombination...
  101. ncbi Expression of MBNL and CELF mRNA transcripts in muscles with myotonic dystrophy
    Yuriko Nezu
    Department of Life Sciences, Graduate School of Arts and Sciences, The University of Tokyo, Tokyo, Japan
    Neuromuscul Disord 17:306-12. 2007
    ..Our results suggest that the expression and stability of the mRNA for these RNA-binding proteins are unaffected in DM1...

Research Grants69

  1. Myotonic Dystrophy Type 2
    Lubov Timchenko; Fiscal Year: 2009
    ..CUG repeat expansion affects two RNA-binding proteins, CUGBP1 and MNBL...
  2. Targeting a Toxic RNA with Small Molecules
    JOHN A BERGLUND; Fiscal Year: 2010
    ..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...
  3. 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 ..
  4. RNA mediated Neurotoxicity in Huntington's Disease-Like 2 and Huntington Disease
    DOBRILA DODA RUDNICKI; Fiscal Year: 2010
    ..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...
  5. Novel Regulators of T Cell mRNA Decay
    Paul Bohjanen; Fiscal Year: 2009
    ..Specific aim 1 is to characterize the role of the GU-rich element (GRE) and the CUG-binding protein 1 (CUGBP1) in regulating T cell mRNA decay...
  6. MOLECULAR PATHOGENESIS OF MYOTONIC DYSTROPHY
    THOMAS ALEXANDER COOPER; Fiscal Year: 2010
    ..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...
  7. MOLECULAR MECHANISMS OF MYOTONIC DYSTROPHY
    Lubov Timchenko; Fiscal Year: 2009
    ..is mainly mediated by untranslated RNA CUG repeats which misregulate two families of RNA CUG-binding proteins, CUGBP1 and MBNL. In DM1, CUG repeats increase protein levels of CUGBP1...
  8. MOLECULAR MECHANISMS OF MYOTONIC DYSTROPHY
    Lubov T Timchenko; Fiscal Year: 2010
    ..is mainly mediated by untranslated RNA CUG repeats which misregulate two families of RNA CUG-binding proteins, CUGBP1 and MBNL. In DM1, CUG repeats increase protein levels of CUGBP1...
  9. Novel Regulators of T Cell mRNA Decay
    Paul R Bohjanen; Fiscal Year: 2010
    ..Specific aim 1 is to characterize the role of the GU-rich element (GRE) and the CUG-binding protein 1 (CUGBP1) in regulating T cell mRNA decay...
  10. Novel Regulators of T Cell mRNA Decay
    Paul R Bohjanen; Fiscal Year: 2010
    ..Specific aim 1 is to characterize the role of the GU-rich element (GRE) and the CUG-binding protein 1 (CUGBP1) in regulating T cell mRNA decay...
  11. MOLECULAR MECHANISMS OF MYOTONIC DYSTROPHY
    Lubov Timchenko; Fiscal Year: 2006
    ..The failure of DM cells to differentiate is accompanying with a failure to accumulate CUGBP1 in cytoplasm...
  12. Targeting CUG Expansions for the Treatment of Myotonic Dystrophy
    Steven C Zimmerman; Fiscal Year: 2010
    ..repeat RNA sequesters MBNL1, a regulator of alternative splicing, and indirectly increases levels of the protein CUGBP. In the parent proposal we are using rational design, computational methods, and high throughput screening to ..
  13. Effect of Aging on Preadipocyte Differentiation
    James Kirkland; Fiscal Year: 2005
    ..We found that CUGBP, a protein that causes LIP to be translated from C/EBPbeta mRNA, increases with aging and TNFalpha treatment...
  14. 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...
  15. MOLECULAR MECHANISMS OF MYOTONIC DYSTROPHY
    Lubov Timchenko; Fiscal Year: 2001
    ..Stable clonal cell lines containing over-expressed (CUG)n repeats or over-expressed CUG-BP/Nab50 will be used to determine whether alteration of CUG-BP/Nab50 protein alters the expression of Mt-PK mRNA from the ..
  16. Molecular Basis of rCGG-Mediated Neurodegeneration
    DAVID LOREN NELSON; Fiscal Year: 2010
    ..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 ..
  17. RNA DOMINANCE IN HUMAN DISEASE
    MAURICE SCOTT SWANSON; Fiscal Year: 2010
    ..because they alter the activities of two families of alternative splicing factors, the muscleblind-like (MBNL) and CUGBP/ETR-3-like factor (CELF) proteins...
  18. Molecular Basis of rCGG-Mediated Neurodegeneration
    DAVID LOREN NELSON; Fiscal Year: 2010
    ..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 ..
  19. MOLECULAR PATHOGENESIS OF MYOTONIC DYSTROPHY
    Thomas Cooper; Fiscal Year: 2003
    ..It will also establish experimental systems to investigate the basis of a novel pathogenic mechanism and ultimately, test means of therapeutic intervention. ..
  20. TROPONIN T ALTERNATIVE SPLICING IN EMBRYONIC HEART
    THOMAS ALEXANDER COOPER; Fiscal Year: 2010
    ..We will continue our analysis of CUGBP and ETR-3 like factors (CELF) and muscleblind-like (MBNL) protein families as direct regulators of alternative ..
  21. TROPONIN T ALTERNATIVE SPLICING IN EMBRYONIC HEART
    Thomas Cooper; Fiscal Year: 2007
    ..This investigation will enhance the understanding of this critical point of gene regulation and establish a foundation to develop approaches to reverse or circumvent pathological processes. ..
  22. MOLECULAR MECHANISMS OF MYOTONIC DYSTROPHY
    JOHN BERGLUND; Fiscal Year: 2009
    ..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. ..
  23. MOLECULAR MECHANISMS OF MYOTONIC DYSTROPHY
    JOHN A BERGLUND; Fiscal Year: 2010
    ..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. ..
  24. MOLECULAR MECHANISMS OF MYOTONIC DYSTROPHY
    JOHN BERGLUND; Fiscal Year: 2009
    ..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. ..
  25. Effect of Aging on Preadipocyte Differentiation
    James Kirkland; Fiscal Year: 2009
    ..animals, increased TNF1 release by preadipocytes with aging, and stress-responsive, anti-adipogenic regulators (CUGBP, CHOP) all impede adipogenesis in old rats...
  26. Effect of Aging on Preadipocyte Differentiation
    James Kirkland; Fiscal Year: 2009
    ..animals, increased TNF1 release by preadipocytes with aging, and stress-responsive, anti-adipogenic regulators (CUGBP, CHOP) all impede adipogenesis in old rats...
  27. Effect of Aging on Preadipocyte Differentiation
    James L Kirkland; Fiscal Year: 2010
    ..animals, increased TNF1 release by preadipocytes with aging, and stress-responsive, anti-adipogenic regulators (CUGBP, CHOP) all impede adipogenesis in old rats...
  28. ABERRANT RNA PROCESSING IN HUMAN DEVELOPMENT
    Adil Shamoo; Fiscal Year: 2002
    ..abstract_text> ..