Wilson Disease

Wilson disease is a genetic disease that prevents the body from removing extra copper. Normally, the liver filters extra copper and releases it into bile. In Wilson disease, the liver does not filter copper correctly and copper builds up in the liver, brain, eyes, and other organs. Wilson disease is caused by an inherited autosomal recessive mutation, or change, in the ATP7B gene. It is named after Samuel Alexander Kinnier Wilson (1878-1937), the British neurologist who first described the condition in 1912. Wilson's disease is a Copper deficiency in humans is rare.1 Toxicity from dietary copper is encountered in humans only in Wilson's disease, a hereditary metabolic disorder in which copper accumulates in body tissues.