Activation of liver X receptor/retinoid X receptor pathway ameliorates liver disease in Atp7B−/− (Wilson disease) mice
James P. Hamilton, Lahari Koganti, Abigael Muchenditsi, Venkata S. Pendyala, David Huso, Joseph Hankin, Robert C. Murphy, Dominik Huster, Uta Merle, Christopher Mangels, Nan Yang, James J. Potter, Esteban Mezey, Svetlana Lutsenko – 17 December 2015 – Wilson disease (WD) is a hepatoneurological disorder caused by mutations in the copper‐transporter, ATP7B. Copper accumulation in the liver is a hallmark of WD. Current therapy is based on copper chelation, which decreases the manifestations of liver disease, but often worsens neurological symptoms.