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Proteomic analysis of mouse models of Niemann-Pick C disease reveals alterations in the steady-state levels of lysosomal proteins within the brain

Niemann-Pick C disease (NPC) is a neurodegenerative lysosomal disorder characterized by storage of cholesterol and other lipids caused by defects in NPC1, a transmembrane protein involved in cholesterol export from the lysosome, or NPC2, an intralysosomal cholesterol transport protein. Alterations i...

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Bibliografiske detaljer
Main Authors: Sleat, David E., Wiseman, Jennifer A., Sohar, Istvan, El –Banna, Mukarram, Zheng, Haiyan, Moore, Dirk F., Lobel, Peter
Format: Artigo
Sprog:Inglês
Udgivet: 2012
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3651901/
https://ncbi.nlm.nih.gov/pubmed/23070805
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/pmic.201200205
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