rbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences
Myotonic dystrophy (DM) type 2 is a neuromuscular pathology caused by large expansions of CCTG repeats. Here the authors find that rbFOX1 RNA binding protein binds to CCUG RNA repeats and competes with MBNL1 for the binding to CCUG repeats, releasing MBNL1 from sequestration in DM2 muscle cells.
| Main Authors: | , , , , , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Nature Portfolio
2018-05-01
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| Series: | Nature Communications |
| Online Access: | https://doi.org/10.1038/s41467-018-04370-x |
| _version_ | 1818399181224542208 |
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| author | Chantal Sellier Estefanía Cerro-Herreros Markus Blatter Fernande Freyermuth Angeline Gaucherot Frank Ruffenach Partha Sarkar Jack Puymirat Bjarne Udd John W. Day Giovanni Meola Guillaume Bassez Harutoshi Fujimura Masanori P. Takahashi Benedikt Schoser Denis Furling Ruben Artero Frédéric H. T. Allain Beatriz Llamusi Nicolas Charlet-Berguerand |
| author_facet | Chantal Sellier Estefanía Cerro-Herreros Markus Blatter Fernande Freyermuth Angeline Gaucherot Frank Ruffenach Partha Sarkar Jack Puymirat Bjarne Udd John W. Day Giovanni Meola Guillaume Bassez Harutoshi Fujimura Masanori P. Takahashi Benedikt Schoser Denis Furling Ruben Artero Frédéric H. T. Allain Beatriz Llamusi Nicolas Charlet-Berguerand |
| author_sort | Chantal Sellier |
| collection | DOAJ |
| description | Myotonic dystrophy (DM) type 2 is a neuromuscular pathology caused by large expansions of CCTG repeats. Here the authors find that rbFOX1 RNA binding protein binds to CCUG RNA repeats and competes with MBNL1 for the binding to CCUG repeats, releasing MBNL1 from sequestration in DM2 muscle cells. |
| first_indexed | 2024-12-14T07:16:36Z |
| format | Article |
| id | doaj.art-cc4a1e87ad4646d6a2914e7d2348f083 |
| institution | Directory Open Access Journal |
| issn | 2041-1723 |
| language | English |
| last_indexed | 2024-12-14T07:16:36Z |
| publishDate | 2018-05-01 |
| publisher | Nature Portfolio |
| record_format | Article |
| series | Nature Communications |
| spelling | doaj.art-cc4a1e87ad4646d6a2914e7d2348f0832022-12-21T23:11:42ZengNature PortfolioNature Communications2041-17232018-05-019111510.1038/s41467-018-04370-xrbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differencesChantal Sellier0Estefanía Cerro-Herreros1Markus Blatter2Fernande Freyermuth3Angeline Gaucherot4Frank Ruffenach5Partha Sarkar6Jack Puymirat7Bjarne Udd8John W. Day9Giovanni Meola10Guillaume Bassez11Harutoshi Fujimura12Masanori P. Takahashi13Benedikt Schoser14Denis Furling15Ruben Artero16Frédéric H. T. Allain17Beatriz Llamusi18Nicolas Charlet-Berguerand19IGBMC, INSERM U964, CNRS UMR7104, University of StrasbourgTranslational Genomics Group, Interdisciplinary Research Structure for Biotechnology and Biomedicine BIOTECMED, University of ValenciaInstitute for Molecular Biology and Biophysics, Swiss Federal Institute of Technology (ETH) ZurichIGBMC, INSERM U964, CNRS UMR7104, University of StrasbourgIGBMC, INSERM U964, CNRS UMR7104, University of StrasbourgIGBMC, INSERM U964, CNRS UMR7104, University of StrasbourgDepartment of Neurology, University of Texas Medical BranchHuman Genetics Research Unit, Laval University, CHUQ, Ste-FoyNeuromuscular Research Center, Tampere University HospitalDepartment of Neurology, Stanford UniversityDepartment of Biomedical Sciences for Health, University of MilanSorbonne Université, Inserm, Association Institut de Myologie, Center of Research in MyologyDepartment of Neurology, Toneyama National HospitalDepartment of Neurology, Osaka University Graduate School of MedicineFriedrich-Baur-Institute, Department of Neurology, Ludwig Maximilian UniversitySorbonne Université, Inserm, Association Institut de Myologie, Center of Research in MyologyTranslational Genomics Group, Interdisciplinary Research Structure for Biotechnology and Biomedicine BIOTECMED, University of ValenciaInstitute for Molecular Biology and Biophysics, Swiss Federal Institute of Technology (ETH) ZurichTranslational Genomics Group, Interdisciplinary Research Structure for Biotechnology and Biomedicine BIOTECMED, University of ValenciaIGBMC, INSERM U964, CNRS UMR7104, University of StrasbourgMyotonic dystrophy (DM) type 2 is a neuromuscular pathology caused by large expansions of CCTG repeats. Here the authors find that rbFOX1 RNA binding protein binds to CCUG RNA repeats and competes with MBNL1 for the binding to CCUG repeats, releasing MBNL1 from sequestration in DM2 muscle cells.https://doi.org/10.1038/s41467-018-04370-x |
| spellingShingle | Chantal Sellier Estefanía Cerro-Herreros Markus Blatter Fernande Freyermuth Angeline Gaucherot Frank Ruffenach Partha Sarkar Jack Puymirat Bjarne Udd John W. Day Giovanni Meola Guillaume Bassez Harutoshi Fujimura Masanori P. Takahashi Benedikt Schoser Denis Furling Ruben Artero Frédéric H. T. Allain Beatriz Llamusi Nicolas Charlet-Berguerand rbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences Nature Communications |
| title | rbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences |
| title_full | rbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences |
| title_fullStr | rbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences |
| title_full_unstemmed | rbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences |
| title_short | rbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences |
| title_sort | rbfox1 mbnl1 competition for ccug rna repeats binding contributes to myotonic dystrophy type 1 type 2 differences |
| url | https://doi.org/10.1038/s41467-018-04370-x |
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