Cells of Matter—In Vitro Models for Myotonic Dystrophy

by Magdalena Matloka, Arnaud F. Klein, Frédérique Rau, Denis Furling
Published 2018-05-01

A CRISPR-Cas13a Based Strategy That Tracks and Degrades Toxic RNA in Myotonic Dystrophy Type 1

by Nan Zhang, Brittani Bewick, Guangbin Xia, Denis Furling, Tetsuo Ashizawa
Published 2020-12-01

Abnormal prostaglandin E2 production blocks myogenic differentiation in myotonic dystrophy

by Daniel Beaulieu, Philippe Thebault, Richard Pelletier, Pierre Chapdelaine, Mark Tarnopolsky, Denis Furling, Jack Puymirat
Published 2012-01-01

The beneficial effect of chronic muscular exercise on muscle fragility is increased by Prox1 gene transfer in dystrophic mdx muscle

by Alexandra Monceau, Clément Delacroix, Mégane Lemaitre, Gaelle Revet, Denis Furling, Onnik Agbulut, Arnaud Klein, Arnaud Ferry
Published 2022-01-01

The beneficial effect of chronic muscular exercise on muscle fragility is increased by Prox1 gene transfer in dystrophic mdx muscle.

by Alexandra Monceau, Clément Delacroix, Mégane Lemaitre, Gaelle Revet, Denis Furling, Onnik Agbulut, Arnaud Klein, Arnaud Ferry
Published 2022-01-01

RBFOX1 cooperates with MBNL1 to control splicing in muscle, including events altered in myotonic dystrophy type 1.

by Roscoe Klinck, Angélique Fourrier, Philippe Thibault, Johanne Toutant, Mathieu Durand, Elvy Lapointe, Marie-Laure Caillet-Boudin, Nicolas Sergeant, Geneviève Gourdon, Giovanni Meola, Denis Furling, Jack Puymirat, Benoit Chabot
Published 2014-01-01

Specific DMPK-promoter targeting by CRISPRi reverses myotonic dystrophy type 1-associated defects in patient muscle cells

by Florent Porquet, Lin Weidong, Kévin Jehasse, Hélène Gazon, Maria Kondili, Silvia Blacher, Laurent Massotte, Emmannuel Di Valentin, Denis Furling, Nicolas Albert Gillet, Arnaud François Klein, Vincent Seutin, Luc Willems
Published 2023-06-01

Pluripotent Stem Cell-Based Drug Screening Reveals Cardiac Glycosides as Modulators of Myotonic Dystrophy Type 1

by Yves Maury, Pauline Poydenot, Benjamin Brinon, Lea Lesueur, Jacqueline Gide, Sylvain Roquevière, Julien Côme, Hélène Polvèche, Didier Auboeuf, Jérome Alexandre Denis, Geneviève Pietu, Denis Furling, Marc Lechuga, Sandrine Baghdoyan, Marc Peschanski, Cécile Martinat
Published 2019-01-01

Dysregulation of Circular RNAs in Myotonic Dystrophy Type 1

by Christine Voellenkle, Alessandra Perfetti, Matteo Carrara, Paola Fuschi, Laura Valentina Renna, Marialucia Longo, Simona Baghai Sain, Rosanna Cardani, Rea Valaperta, Gabriella Silvestri, Ivano Legnini, Irene Bozzoni, Denis Furling, Carlo Gaetano, Germana Falcone, Giovanni Meola, Fabio Martelli
Published 2019-04-01

Molecular, physiological, and motor performance defects in DMSXL mice carrying >1,000 CTG repeats from the human DM1 locus.

by Aline Huguet, Fadia Medja, Annie Nicole, Alban Vignaud, Céline Guiraud-Dogan, Arnaud Ferry, Valérie Decostre, Jean-Yves Hogrel, Friedrich Metzger, Andreas Hoeflich, Martin Baraibar, Mário Gomes-Pereira, Jack Puymirat, Guillaume Bassez, Denis Furling, Arnold Munnich, Geneviève Gourdon
Published 2012-01-01

In Vitro and In Vivo Modulation of Alternative Splicing by the Biguanide Metformin

by Delphine Laustriat, Jacqueline Gide, Laetitia Barrault, Emilie Chautard, Clara Benoit, Didier Auboeuf, Anne Boland, Christophe Battail, François Artiguenave, Jean-François Deleuze, Paule Bénit, Pierre Rustin, Sylvia Franc, Guillaume Charpentier, Denis Furling, Guillaume Bassez, Xavier Nissan, Cécile Martinat, Marc Peschanski, Sandrine Baghdoyan
Published 2015-01-01

Immortalized human myotonic dystrophy muscle cell lines to assess therapeutic compounds

by Ludovic Arandel, Micaela Polay Espinoza, Magdalena Matloka, Audrey Bazinet, Damily De Dea Diniz, Naïra Naouar, Frédérique Rau, Arnaud Jollet, Frédérique Edom-Vovard, Kamel Mamchaoui, Mark Tarnopolsky, Jack Puymirat, Christophe Battail, Anne Boland, Jean-Francois Deleuze, Vincent Mouly, Arnaud F. Klein, Denis Furling
Published 2017-04-01

Downregulation of the Glial GLT1 Glutamate Transporter and Purkinje Cell Dysfunction in a Mouse Model of Myotonic Dystrophy

by Géraldine Sicot, Laurent Servais, Diana M. Dinca, Axelle Leroy, Cynthia Prigogine, Fadia Medja, Sandra O. Braz, Aline Huguet-Lachon, Cerina Chhuon, Annie Nicole, Noëmy Gueriba, Ruan Oliveira, Bernard Dan, Denis Furling, Maurice S. Swanson, Ida Chiara Guerrera, Guy Cheron, Geneviève Gourdon, Mário Gomes-Pereira
Published 2017-06-01

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

by 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
Published 2018-05-01

Splicing misregulation of SCN5A contributes to cardiac-conduction delay and heart arrhythmia in myotonic dystrophy

by Fernande Freyermuth, Frédérique Rau, Yosuke Kokunai, Thomas Linke, Chantal Sellier, Masayuki Nakamori, Yoshihiro Kino, Ludovic Arandel, Arnaud Jollet, Christelle Thibault, Muriel Philipps, Serge Vicaire, Bernard Jost, Bjarne Udd, John W. Day, Denis Duboc, Karim Wahbi, Tsuyoshi Matsumura, Harutoshi Fujimura, Hideki Mochizuki, François Deryckere, Takashi Kimura, Nobuyuki Nukina, Shoichi Ishiura, Vincent Lacroix, Amandine Campan-Fournier, Vincent Navratil, Emilie Chautard, Didier Auboeuf, Minoru Horie, Keiji Imoto, Kuang-Yung Lee, Maurice S. Swanson, Adolfo Lopez de Munain, Shin Inada, Hideki Itoh, Kazuo Nakazawa, Takashi Ashihara, Eric Wang, Thomas Zimmer, Denis Furling, Masanori P. Takahashi, Nicolas Charlet-Berguerand
Published 2016-04-01