Inhibition of β-Glucocerebrosidase Activity Preserves Motor Unit Integrity in a Mouse Model of Amyotrophic Lateral Sclerosis

Inhibition of β-Glucocerebrosidase Activity Preserves Motor Unit Integrity in a Mouse Model of Amyotrophic Lateral Sclerosis

Abstract Recent metabolomic reports connect dysregulation of glycosphingolipids, particularly ceramide and glucosylceramide, to neurodegeneration and to motor unit dismantling in amyotrophic lateral sclerosis at late disease stage. We report here altered levels of gangliosides in the cerebrospinal f...

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Bibliographic Details
Main Authors: Alexandre Henriques, Mylene Huebecker, Hélène Blasco, Céline Keime, Christian R. Andres, Philippe Corcia, David A. Priestman, Frances M. Platt, Michael Spedding, Jean-Philippe Loeffler
Format: Article
Language:English
Published: Nature Publishing Group 2017-07-01
Series:Scientific Reports
Online Access:https://doi.org/10.1038/s41598-017-05313-0

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https://doi.org/10.1038/s41598-017-05313-0

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