Copper and Brain Function

Abstract

Copper is an essential trace element which forms an integral component of copper-dependent enzymes, including cytochrome c oxidase, dopamine β hydroxylase, lysyl oxidase, Cu/Zn superoxide dismutase, tyrosinase and ceruloplasmin. Copper deficiency results in decrease of these enzyme activities. Menkes disease, a genetic disorder of a　copper-transporting ATPase deficiency, shows the typical manifestations of copper deficiency, including neurological disturbances, hypothermia, hair abnormalities and arterial abnormalities. These disorders are very severe and thus most patients die by the age of 3 years. In patients with mild type of Menkes disease, the characteristic neurological finding is ataxia, suggesting that mild copper deficiency causes ataxia.

Copper accumulation in the brain also damages the brain. In Wilson disease, another genetic disorder of copper metabolism, copper accumulates in the brain, resulting in neurological disturbances, including extrapyramidal symptoms and psycological disorders. In familial amyotrophic lateral sclerosis, a fatal progressive motor neuron disease, also, copper toxicity in motor neurons is suspected to contribute to the pathogenesis.