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miR-23b and miR-218 silencing increase Muscleblind-like expression and alleviate myotonic dystrophy phenotypes in mammalian models

Depletion of the splicing factors MBNL 1 and 2 causes myotonic dystrophy. Here, the authors show that miR-23b and miR-218 target MBNL proteins, and that antagonists to these miRNAs rescue mis-splicing events in myoblasts and boost MBNL expression and rescue pathology in mouse models.

Estefania Cerro-Herreros
Maria Sabater-Arcis
Ruben Artero
ResearchOpen Access26 Jun 2018
Nature Communications

Volume: 9, P: 1-13
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.

Chantal Sellier
Estefanía Cerro-Herreros
Nicolas Charlet-Berguerand
ResearchOpen Access22 May 2018
Nature Communications

Volume: 9, P: 1-15
Use of HSA^LR female mice as a model for the study of myotonic dystrophy type I

The authors compare muscle function, histology and molecular markers, and biochemical plasma profiles in male and female HSA^LR mice to identify potential sex differences in this mouse model of myotonic dystrophy type I.

Marc Carrascosa-Sàez
Anna Colom-Rodrigo
Ruben Artero
ResearchOpen Access27 Feb 2025
Lab Animal

Volume: 54, P: 92-102
Defined d-hexapeptides bind CUG repeats and rescue phenotypes of myotonic dystrophy myotubes in a Drosophila model of the disease

Anna Rapisarda
Ariadna Bargiela
Manuel Perez-Alonso
ResearchOpen Access30 Sept 2021
Scientific Reports

Volume: 11, P: 1-15
Rabphilin silencing causes dilated cardiomyopathy in a Drosophila model of nephrocyte damage

Estela Selma-Soriano
Carlos Casillas-Serra
Josep Redón
ResearchOpen Access27 Jul 2021
Scientific Reports

Volume: 11, P: 1-13
Derepressing muscleblind expression by miRNA sponges ameliorates myotonic dystrophy-like phenotypes in Drosophila

Estefania Cerro-Herreros
Juan M. Fernandez-Costa
Ruben Artero
ResearchOpen Access02 Nov 2016
Scientific Reports

Volume: 6, P: 1-13
Expanded CCUG repeat RNA expression in Drosophila heart and muscle trigger Myotonic Dystrophy type 1-like phenotypes and activate autophagocytosis genes

Estefania Cerro-Herreros
Mouli Chakraborty
Beatriz Llamusí
ResearchOpen Access06 Jun 2017
Scientific Reports

Volume: 7, P: 1-13

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miR-23b and miR-218 silencing increase Muscleblind-like expression and alleviate myotonic dystrophy phenotypes in mammalian models

rbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences

Use of HSA^LR female mice as a model for the study of myotonic dystrophy type I

Defined d-hexapeptides bind CUG repeats and rescue phenotypes of myotonic dystrophy myotubes in a Drosophila model of the disease

Rabphilin silencing causes dilated cardiomyopathy in a Drosophila model of nephrocyte damage

Derepressing muscleblind expression by miRNA sponges ameliorates myotonic dystrophy-like phenotypes in Drosophila

Expanded CCUG repeat RNA expression in Drosophila heart and muscle trigger Myotonic Dystrophy type 1-like phenotypes and activate autophagocytosis genes

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miR-23b and miR-218 silencing increase Muscleblind-like expression and alleviate myotonic dystrophy phenotypes in mammalian models

rbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences

Use of HSALR female mice as a model for the study of myotonic dystrophy type I

Defined d-hexapeptides bind CUG repeats and rescue phenotypes of myotonic dystrophy myotubes in a Drosophila model of the disease

Rabphilin silencing causes dilated cardiomyopathy in a Drosophila model of nephrocyte damage

Derepressing muscleblind expression by miRNA sponges ameliorates myotonic dystrophy-like phenotypes in Drosophila

Expanded CCUG repeat RNA expression in Drosophila heart and muscle trigger Myotonic Dystrophy type 1-like phenotypes and activate autophagocytosis genes

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Quick links

Use of HSA^LR female mice as a model for the study of myotonic dystrophy type I