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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Detalles Bibliográficos
Autores principales: Lecommandeur, Emmanuelle, Cachón-González, Maria Begoña, Boddie, Susannah, McNally, Ben D., Nicholls, Andrew W., Cox, Timothy M., Griffin, Julian L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7823728/
https://www.ncbi.nlm.nih.gov/pubmed/33396723
http://dx.doi.org/10.3390/metabo11010018

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7823728/
https://www.ncbi.nlm.nih.gov/pubmed/33396723
http://dx.doi.org/10.3390/metabo11010018

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