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doi: 10.1097/00006454-200103000-00016 80 HegstadK.MylvaganamH.JaniceJ.JosefsenE.SivertsenA.SkaareD

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Cell Calcium 49, 296305

This transporter localizes to the trans-Golgi network or late endosomes under basal conditions, but it relocalizes in a copper-dependent manner to a subapical, vesicular compartment, which facilitates both copper incorporation into ceruloplasmin and biliary copper excretion via lysosomes.180 Apart from copper, Long-Evans Cinnamon rats also have abnormal hepatic accumulation of iron.181 Chronic copper/iron accumulation in these rats leads to a 50% increase in lipid peroxidation, presumably due to copper/iron capability to induce oxidative stress by generating ROS via both mitochondrial dysfunction182 and Fenton-type, copper/iron-catalyzed Haber-Weiss reaction.183,184 These mutant rats exhibit a reduced basal bile salt-biliary excretion compared with normal, Long-Evans Agouti rats