Integrated gut microbiome and lipidomic analyses in animal models of Wilson disease reveal a role of intestine ATP7B in copper-related metabolic dysregulation

Wilson disease is due to hepatic copper accumulation in the liver and brain, but the role of organ-specific ATP7B copper transporter mutations on systemic manifestations of the disease is unknown.Gut microbiome and lipidome profiles distinguish mouse models of Wilson disease from mice with normal copper metabolism. An intestine-specific Atp7b knockout reveals affected lipid processing independent of liver disease.Wilson's disease should be considered and treated as a systemic disease. Organ-specific ATP7B mutations and lipid and energy metabolism should be considered when designing anti-copper treatments.