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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 fluid of amyotrophic lateral sclerosis patients in early disease stage. Conduritol B epoxide is an inhibitor of acid beta-glucosidase, and lowers glucosylceramide degradation. Glucosylceramide is the precur...

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Publication status:
Published
Peer review status:
Peer reviewed
Version:
Publisher's version

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Publisher copy:
10.1038/s41598-017-05313-0

Authors


Henriques, A More by this author
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Department:
Oxford, MSD, Pharmacology
Andres, CR More by this author
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Funding agency for:
Huebecker, M
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Funding agency for:
Platt, FM
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Funding agency for:
Platt, FM
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Grant:
Health Seventh Framework Programme under grant agreement No. 259867 (Euro-MOTOR, JPL)
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Publisher:
Springer Nature Publisher's website
Journal:
Scientific Reports Journal website
Volume:
7
Pages:
Article: 5235
Publication date:
2017-07-12
Acceptance date:
2017-05-26
DOI:
ISSN:
2045-2322
Pubs id:
pubs:697564
URN:
uri:7f08b052-98b9-447a-853f-a17e0110ae92
UUID:
uuid:7f08b052-98b9-447a-853f-a17e0110ae92
Local pid:
pubs:697564

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