Weekly Cyclodextrin Administration Normalizes Cholesterol Metabolism in Nearly Every Organ of the Niemann-Pick Type C1 Mouse and Markedly Prolongs Life

Niemann-Pick type C1 disease arises from a mutation inactivating NPC1 protein that normally moves unesterified cholesterol from the late endosomal/lysosomal complex of cells to the cytosolic compartment for processing. As a result, cholesterol accumulates in every tissue of the body causing liver, l...

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Bibliografiska uppgifter
Huvudupphovsmän: Ramirez, Charina M., Liu, Benny, Taylor, Anna M., Repa, Joyce J., Burns, Dennis K., Weinberg, Arthur G., Turley, Stephen D., Dietschy, John M.
Materialtyp: Artigo
Språk:Inglês
Publicerad: 2010
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Article
Länkar:https://ncbi.nlm.nih.gov/pmc/articles/PMC3065173/
https://ncbi.nlm.nih.gov/pubmed/20581737
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1203/PDR.0b013e3181ee4dd2
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