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.
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2018
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oai:doaj.org-article:cc4a1e87ad4646d6a2914e7d2348f0832021-12-02T13:27:31ZrbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences10.1038/s41467-018-04370-x2041-1723https://doaj.org/article/cc4a1e87ad4646d6a2914e7d2348f0832018-05-01T00:00:00Zhttps://doi.org/10.1038/s41467-018-04370-xhttps://doaj.org/toc/2041-1723Myotonic 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.Chantal SellierEstefanía Cerro-HerrerosMarkus BlatterFernande FreyermuthAngeline GaucherotFrank RuffenachPartha SarkarJack PuymiratBjarne UddJohn W. DayGiovanni MeolaGuillaume BassezHarutoshi FujimuraMasanori P. TakahashiBenedikt SchoserDenis FurlingRuben ArteroFrédéric H. T. AllainBeatriz LlamusiNicolas Charlet-BerguerandNature PortfolioarticleScienceQENNature Communications, Vol 9, Iss 1, Pp 1-15 (2018) |
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Science Q 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 |
| 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. |
| format |
article |
| 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 |
| title |
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_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_sort |
rbfox1/mbnl1 competition for ccug rna repeats binding contributes to myotonic dystrophy type 1/type 2 differences |
| publisher |
Nature Portfolio |
| publishDate |
2018 |
| url |
https://doaj.org/article/cc4a1e87ad4646d6a2914e7d2348f083 |
| work_keys_str_mv |
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