Copper Deficiency and the Clinical Practice

In 1993, the candidate genes for Menkes disease and Wilson disease were cloned, and remarkable progress has been made in the study of copper metabolism during the past 10 years. The proteins induced by the ATP7A and ATP7B genes are highly homologous. Both are P-type ATP-related coppertransporter membrane proteins, and control cellular copper transport. Recently, three chaperones supplying copper to the intracellular copper-requiring enzymes were discovered, and the physiology of intracellular copper metabolism is becoming more and more clear. Numerous copper-requiring enzymes are present in the body, therefore, copper deficiency may lead to various disorders. Menkes disease is well-known as an inherited disorder of copper transport from the intestine resulting in copper deficiency. In regard to acquired copper deficiency, nutritional deficiency is probably the most common cause, and may be seen in malnourished low-birth-weight infants, newborns, and small infants. Copper deficiency has also been reported to develop after gastrointestinal surgery, intractable diarrhea, and prolonged parenteral or enteral nutrition. In this article, I present a review of copper deficiency and its treatment.