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

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 fluid of a...

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Detalles Bibliográficos
Autores principales: Henriques, Alexandre, Huebecker, Mylene, Blasco, Hélène, Keime, Céline, Andres, Christian R., Corcia, Philippe, Priestman, David A., Platt, Frances M., Spedding, Michael, Loeffler, Jean-Philippe
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5507914/
https://www.ncbi.nlm.nih.gov/pubmed/28701774
http://dx.doi.org/10.1038/s41598-017-05313-0