Copper(I)-binding properties of de-coppering drugs for the treatment of Wilson disease. α-Lipoic acid as a potential anti-copper agent

• Published in: Scientific reports Vol. 8; no. 1; pp. 1463 - 9
• Main Authors: Smirnova, Julia, Kabin, Ekaterina, Järving, Ivar, Bragina, Olga, Tõugu, Vello, Plitz, Thomas, Palumaa, Peep
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 23.01.2018; Nature Publishing Group; Nature Portfolio
• Subjects: 631/154; 692/4017; 82/58; Adenosine triphosphatase; Affinity; Autosomal recessive inheritance; Biological activity; Cell culture; Chelation; Copper; Copper-transporting ATPase; Cytochrome-c oxidase; Drugs; Excretion; Genetic disorders; Hereditary diseases; Humanities and Social Sciences; Lipoic acid; Liver diseases; Metallothionein; multidisciplinary; Mutation; Propanol; Science; Science (multidisciplinary); Sulfur; Toxicity; Wilson's disease

Wilson disease is an autosomal recessive genetic disorder caused by loss-of-function mutations in the P-type copper ATPase, ATP7B , which leads to toxic accumulation of copper mainly in the liver and brain. Wilson disease is treatable, primarily by copper-chelation therapy, which promotes copper exc...