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- W2897344729 endingPage "112" @default.
- W2897344729 startingPage "98" @default.
- W2897344729 abstract "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." @default.
- W2897344729 created "2018-10-26" @default.
- W2897344729 creator A5000627885 @default.
- W2897344729 creator A5003730884 @default.
- W2897344729 creator A5029983216 @default.
- W2897344729 creator A5080871218 @default.
- W2897344729 date "2019-01-01" @default.
- W2897344729 modified "2023-09-24" @default.
- W2897344729 title "Chelating principles in Menkes and Wilson diseases" @default.
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