Intracellular trafficking of the free cholesterol derived from LDL cholesteryl ester is defective in vivo in Niemann-Pick C disease: insights on normal metabolism of HDL and LDL gained from the NP-C mutation

Intracellular trafficking of the free cholesterol derived from LDL cholesteryl ester is defective in vivo in Niemann-Pick C disease: insights on normal metabolism of HDL and LDL gained from the NP-C mutation

Niemann-Pick C disease (NP-C) is a rare inborn error of metabolism with hepatic involvement and neurological sequelae that usually manifest in childhood. Although in vitro studies have shown that the lysosomal distribution of LDL-derived cholesterol is defective in cultured cells of NP-C subjects, n...

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Bibliographic Details
Main Authors: R D Shamburek, P G Pentchev, L A Zech, J Blanchette-Mackie, E D Carstea, J M VandenBroek, P S Cooper, E B Neufeld, R D Phair, H B Brewer, Jr, R O Brady, C C Schwartz
Format: Article
Language:English
Published: Elsevier 1997-12-01
Series:Journal of Lipid Research
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520300274

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http://www.sciencedirect.com/science/article/pii/S0022227520300274

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