AbstractCopper oxide nanoparticles (CuO-NPs) are well known for their cytotoxicity which in part has been attributed to the release of copper ions from CuO-NPs. As iron-doping has been reported to reduce the susceptibility of CuO-NPs to dissolution, we have compared pure CuO-NPs and CuO-NPs that had been doped with 10% iron (CuO-Fe-NPs) for copper release and for their toxic potential on C6 glioma cells. Physicochemical characterization revealed that dimercaptosuccinate (DMSA)-coated CuO-NPs and CuO-Fe-NPs did not differ in their size or zeta potential. However, the redox activity and liberation of copper ions from CuO-Fe-NPs was substantially slower compared to that from CuO-NPs, as demonstrated by cyclic voltammetry and by the photometric quantification of the copper ion-bathocuproine complex, respectively. Exposure of C6 cells to these NPs caused an almost identical cellular copper accumulation and each of the two types of NPs induced ROS production and cell toxicity. However, the time- and concentration-dependent loss in cell viability was more severe for cells that had been treated with CuO-NPs compared to cells exposed to CuO-Fe-NPs. Copper accumulation and toxicity after exposure to either CuO-NPs or CuO-Fe-NPs was prevented in the presence of copper chelators, while neutralization of the lysosomal pH by bafilomycin A1 prevented toxicity without affecting cellular copper accumulation or ROS production. These data demonstrate that iron-doping does not affect cellular accumulation of CuO-NPs and suggests that the intracellular liberation of copper ions from CuO-NPs is slowed by the iron doping, which in turn lowers the cell toxic potential of iron-doped CuO-NPs.

01 Jan 2019·Journal of Inorganic BiochemistryQ2 · BIOLOGY

Chelating principles in Menkes and Wilson diseases

Q2 · BIOLOGY

Review

Author: Horn, Nina ; Møller, Lisbeth Birk ; Nurchi, Valeria Marina ; Aaseth, Jan

Dysregulation of copper homeostasis in humans is primarily found in two genetic diseases of copper transport, Menkes and Wilson diseases, which show symptoms of copper deficiency or overload, respectively. However, both diseases are copper storage disorders despite completely opposite clinical pictures. Clinically, Menkes disease is characterized by copper deficiency secondary to poor loading of copper-requiring enzymes although sufficient body copper. Copper accumulates in non-hepatic tissues, but is deficient in blood, liver, and brain. In contrast, Wilson disease is characterized by symptoms of copper toxicity secondary to accumulation of copper in several organs most notably brain and liver, and a saturated blood copper pool. It is a challenge to correct copper dyshomeostasis in either disease though copper depletion in Menkes disease is most challenging. Both diseases are caused by defective copper export from distinct cells, and we seek to give new angles and guidelines to improve treatment of these two complementary diseases. Therapy of Menkes disease with copper-histidine, thiocarbamate, nitrilotriacetate or lipoic acid is discussed. In Wilson disease combination of a hydrophilic chelator e.g. trientine or dimercaptosuccinate with a brain shuttle e.g. thiomolybdate or lipoate, is discussed. New chelating principles for copper removal or delivery are outlined.

01 Jan 2017·Intractable & Rare Diseases Research

Epidemiology, diagnosis, and treatment of Wilson's disease

Review

Author: Zhou, Xiaoyan ; Han, Jinxiang ; Cui, Yazhou ; Luan, Jing ; Liu, Jing

Wilson's disease (WD) is an autosomal recessive disease caused by a mutation of the ATP7B gene, resulting in abnormal copper metabolism. The major clinical features of WD include liver disease, neurological disorders, K-F rings, and osteoporosis. The prevalence of WD in China is higher than that in Western countries. Early diagnosis and lifelong treatment will lead to better outcomes. Drugs such as sodium dimercaptosuccinate (Na-DMPS), Zn, and Gandou Decoction can be used to treat WD. Some studies have shown that the combination of traditional Chinese medicine and Western medicine is the best approach to treating WD. In order to identify better treatments, this article describes the specific clinical symptoms of Wilson's disease, its diagnosis, and treatment options.

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Indication	Country/Location	Organization	Date
Hepatolenticular Degeneration	CN	-	30 Apr 1965

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