Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1(−/−) mouse

Niemann-Pick type C disease is largely attributable to an inactivating mutation of NPC1 protein, which normally aids movement of unesterified cholesterol (C) from the endosomal/lysosomal (E/L) compartment to the cytosolic compartment of cells throughout the body. This defect results in activation of...

Disgrifiad llawn

Wedi'i Gadw mewn:
Manylion Llyfryddiaeth
Prif Awduron: Liu, Benny, Turley, Stephen D., Burns, Dennis K., Miller, Anna M., Repa, Joyce J., Dietschy, John M.
Fformat: Artigo
Iaith:Inglês
Cyhoeddwyd: National Academy of Sciences 2009
Pynciau:
Biological Sciences
Mynediad Ar-lein:https://ncbi.nlm.nih.gov/pmc/articles/PMC2650164/
https://ncbi.nlm.nih.gov/pubmed/19171898
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0810895106
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