Decrease in Myelin-Associated Lipids Precedes Neuronal Loss and Glial Activation in the CNS of the Sandhoff Mouse as Determined by Metabolomics

Decrease in Myelin-Associated Lipids Precedes Neuronal Loss and Glial Activation in the CNS of the Sandhoff Mouse as Determined by Metabolomics

Sandhoff disease (SD) is a lysosomal disease caused by mutations in the gene coding for the β subunit of β-hexosaminidase, leading to deficiency in the enzymes β-hexosaminidase (HEX) A and B. SD is characterised by an accumulation of gangliosides and related glycolipids, mainly in the central nervou...

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Bibliographic Details
Main Authors: Emmanuelle Lecommandeur, Maria Begoña Cachón-González, Susannah Boddie, Ben D. McNally, Andrew W. Nicholls, Timothy M. Cox, Julian L. Griffin
Format: Article
Language:English
Published: MDPI AG 2020-12-01
Series:Metabolites
Subjects:
lipidomics
metabolomics
lysosomal disorders
β-hexosaminidase
galactosylceramides
bis(monoacylglycero)phosphates
Online Access:https://www.mdpi.com/2218-1989/11/1/18

Internet

https://www.mdpi.com/2218-1989/11/1/18

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