Article
Methanobactin reverses acute liver failure in a rat model of Wilson disease.
The Journal of clinical investigation - 1 Jul 2016
Lichtmannegger Josef, Leitzinger Christin, Wimmer Ralf, Schmitt Sabine, Schulz Sabine, Kabiri Yaschar, Eberhagen Carola, Rieder Tamara, Janik Dirk, Neff Frauke, Straub Beate K, Schirmacher Peter, DiSpirito Alan A, Bandow Nathan, Baral Bipin S, Flatley Andrew, Kremmer Elisabeth, Denk Gerald, Reiter Florian P, Hohenester Simon, Eckardt-Schupp Friedericke, Dencher Norbert A, Adamski Jerzy, Sauer Vanessa, Niemietz Christoph, Schmidt Hartmut H J, Merle Uta, Gotthardt Daniel Nils, Kroemer Guido, Weiss Karl Heinz, Zischka Hans
Abstract excerpt
In Wilson disease (WD), functional loss of ATPase copper-transporting β (ATP7B) impairs biliary copper excretion, leading to excessive copper accumulation in the liver and fulminant hepatitis. Current US Food and Drug Administration- and European Medicines Agency-approved pharmacological treatments usually fail to restore copper homeostasis in patients with WD who have progressed to acute liver failure, leaving...
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