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Monitoring the itinerary of lysosomal cholesterol in Niemann-Pick Type C1-deficient cells after cyclodextrin treatment

Niemann-Pick disease type C (NPC) disease is a lipid-storage disorder that is caused by mutations in the genes encoding NPC proteins and results in lysosomal cholesterol accumulation. 2-Hydroxypropyl-β-cyclodextrin (CD) has been shown to reduce lysosomal cholesterol levels and enhance sterol homeost...

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Detalles Bibliográficos
Publicado en:J Lipid Res
Main Authors: Feltes, McKenna, Gale, Sarah E., Moores, Samantha, Ory, Daniel S., Schaffer, Jean E.
Formato: Artigo
Idioma:Inglês
Publicado: The American Society for Biochemistry and Molecular Biology 2020
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Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC7053843/
https://ncbi.nlm.nih.gov/pubmed/31988149
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1194/jlr.RA119000571
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