Fig. (2).

Copper metabolism in normal cells versus those affected by Menkes disease.

CTR1, copper transporter 1; ATP7A, copper-transporting P-type ATPase ; ●, copper. Left, copper metabolism in normal cells. Right, copper metabolism in cells affected by Menkes disease. In cells affected by Menkes disease, copper cannot be transported from the cytosol to the Golgi apparatus. As a result, copper accumulates in the cytosol and cannot be excreted from the cells. Copper deficiency in the Golgi apparatus results in a decrease in the activities of secretory copper enzymes such as lysyl oxidase (LOX) and dopamine β-hydroxidase (DBH).