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

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

Brain function is compromised in myotonic dystrophy type 1 (DM1), but the underlying mechanisms are not fully understood. To gain insight into the cellular and molecular pathways primarily affected, we studied a mouse model of DM1 and brains of adult patients. We found pronounced RNA toxicity in the...

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Bibliographic Details
Main Authors: 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
Format: Article
Language:English
Published: Elsevier 2017-06-01
Series:Cell Reports
Subjects:
myotonic dystrophy
unstable microsatellite repeats
brain
cerebellum
neurons
astrocytes
Bergmann glia
GLT1
glutamate
transgenic mice
ceftriaxone
Online Access:http://www.sciencedirect.com/science/article/pii/S2211124717307854

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http://www.sciencedirect.com/science/article/pii/S2211124717307854

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