COPPER DEFICIENCY IN THE DEVELOPING RAT BRAIN: A POSSIBLE MODEL FOR MENKES' STEELY-HAIR DISEASE

Abstract— In comparison to controls, copper-deficient suckling rats showed an appreciable decrease in body growth, a slight decrease in whole brain and cerebellar growth, and a highly significant decrease in myelination based on the activity of cerebellar 2',3'-cyclic nucleotide 3'-phosphohydrolase—a myelin enriched protein. Specific effects of a fivefold reduction in the copper content of brain were seen in a drastic decrease in cerebellar cytochrome c oxidase and smaller but significant drops in cerebellar super-oxide dismutase and brain norepinephrine concn. These observations are discussed with respect to the neuropathology and biochemistry of Menkes' steely-hair disease, a sex-linked recessive disorder in humans characterized by copper deficiency.