Immortalized human myotonic dystrophy muscle cell lines to assess therapeutic compounds

Immortalized human myotonic dystrophy muscle cell lines to assess therapeutic compounds

Myotonic dystrophy type 1 (DM1) and type 2 (DM2) are autosomal dominant neuromuscular diseases caused by microsatellite expansions and belong to the family of RNA-dominant disorders. Availability of cellular models in which the DM mutation is expressed within its natural context is essential to faci...

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Bibliographic Details
Main Authors: 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
Format: Article
Language:English
Published: The Company of Biologists 2017-04-01
Series:Disease Models & Mechanisms
Subjects:
Myotonic dystrophy
Expanded repeats
Muscle cell line
Nuclear aggregates
Alternative splicing
Therapeutic compounds
Online Access:http://dmm.biologists.org/content/10/4/487

Internet

http://dmm.biologists.org/content/10/4/487

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