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Increased copper levels in in vitro and in vivo models of Niemann-Pick C disease

Content Provider	Springer Nature Link
Author	Vázquez, Mary Carmen Martínez, Pablo Alvarez, Alejandra R. González, Mauricio Zanlungo, Silvana
Copyright Year	2012
Abstract	Niemann-Pick type C disease (NPC) is a hereditary neurovisceral atypical lipid storage disorder produced by mutations in the NPC1 and NPC2 genes. The disease is characterized by unesterified cholesterol accumulation in late endosomal/lysosomal compartments and oxidative stress. The most affected tissues are the cerebellum and the liver. The lysotropic drug U18666A (U18) has been widely used as a pharmacological model to induce the NPC phenotype in several cell culture lines. It has already been reported that there is an increase in copper content in hepatoma Hu7 cells treated with U18. We confirmed this result with another human hepatoma cell line, HepG2, treated with U18 and supplemented with copper in the media. However, in mouse hippocampal primary cultures treated under similar conditions, we did not find alterations in copper content. We previously reported increased copper content in the liver of Npc1 −/− mice compared to control animals. Here, we extended the analysis to the copper content in the cerebella, the plasma and the bile of NPC1 deficient mice. We did not observe a significant change in copper content in the cerebella, whereas we found increased copper content in the plasma and decreased copper levels in the bile of Npc1−/− mice. Finally, we also evaluated the plasma content of ceruloplasmin, and we found an increase in this primary copper-binding protein in Npc1 −/− mice. These results indicate cell-type dependence of copper accumulation in NPC disease and suggest that copper transport imbalance may be relevant to the liver pathology observed in NPC disease.
Starting Page	777
Ending Page	786
Page Count	10
File Format	PDF
ISSN	09660844
Journal	BioMetals
Volume Number	25
Issue Number	4
e-ISSN	15728773
Language	English
Publisher	Springer Netherlands
Publisher Date	2012-04-17
Publisher Place	Dordrecht
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Copper Niemann-Pick type C Liver Ceruloplasmin Plant Physiology Microbiology Biochemistry Pharmacology/Toxicology Medicine/Public Health Cell Biology
Content Type	Text
Resource Type	Article
Subject	Metals and Alloys Biochemistry, Genetics and Molecular Biology Biomaterials Agricultural and Biological Sciences

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