Abstract: Menkes disease is a X-linked recessive disorder of brain copper metabolism caused by mutations in an essential mammalian copper transporter gene, ATP7A, affecting approximately 200 to 400 individuals in the United States. Copper replacement therapy has been used to treat Menkes disease. The present disclosure shows that treatment of Menkes disease. The present disclosure shows that treatment of Menkes disease patients with copper histidinate increases survival of the patients.