Copper-binding properties of the N-terminus of the Menkes protein

Copper is an essential element that functions as a cofactor in a wide variety of enzymes including cytochrome C-oxidase, Cu-Zn Superoxide dismutase, lysyl oxidase, amine oxidase and dopamine-ß-hydroxylase. Copper deficiencies are characterised by skeletal abnormalities, severe mental retardation, neurologic degeneration and mortality in extreme cases. These symptoms of deficiency are evident in newborns diagnosed with Menkes disease. The symptoms of Menkes disease result from the decreased activity of copper-dependent enzymes (Kodama, 1993). The redox properties of copper that make it so effective as a cofactor in single electron transfer also contribute to its toxicity when it is present in excess. The Menkes protein is one part of the mechanism by which the human body maintains cellular levels of copper between the limits of deficiency and excess