STUDIES ON COPPER METABOLISM. XIV. COPPER, CERULO-PLASMIN AND OXIDASE ACTIVITY IN SERA OF NORMAL HUMAN SUBJECTS, PREGNANT WOMEN, AND PATIENTS WITH INFECTION, HEPATOLENTICULAR DEGENERATION AND THE NEPHROTIC SYNDROME 1

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Abstract
Total serum Cu, direct-reacting Cu, indirect-reacting Cu, ceruloplasmin and oxidase activity were measured in the sera of 10 normal subjects. The mean values observed were: total serum Cu, 108 [plus or minus] 9 [mu]g/100 ml; direct-reacting Cu, 5 [plus or minus] 6 [mu]g/100 ml; indirect-reacting Cu, 103 [plus or minus] 11 [mu]g/100 ml; ceruloplasmin, 34 [plus or minus] 4 [mu]g/100 ml; oxidase activity, 3.9 [plus or minus] 0.7 [mu][image] O2/ml/hour. Corresponding values in 10 pregnant women in the last trimester of pregnancy were: total serum Cu, 257 [plus or minus] 38 [mu]g/100 ml; direct-reacting Cu, 29 [plus or minus] 13 [mu]g/100 ml; indirect-reacting Cu, 228 [plus or minus] 35 [mu]g/100 ml; ceruloplasmin, 84 [plus or minus] 15 mg/100 ml; oxidase activity, 9.6 [plus or minus] 2.2 [mu]M 02/ml/hour. A high degree of correlation was observed between the total serum Cu, indirect-reacting Cu , ceruloplasmin and oxidase activity. Mean values in 7 patients with chronic infections were: total serum Cu, 192 [mu]g/100 ml; direct-reacting Cu, 17 ug/100 ml: indirect-reacting Cu, 175 [mu]g/100 ml; ceruloplasmin, 68 mg/100 ml; oxidase activity, 8.1 [mu][image] O2/ml/hour. Correlation was high. In 9 patients with the nephrotic syndrome, the total serum Cu values ranged from 129 Mg/100 ml to as low as 36 Mg/100 ml. In those in whom there was hypocupremia, a corresponding reduction in the ceruloplasmin concentration was present. Four of these patients were found to excrete between 46 and 75 mg of cerulo-plasmin/24 hours in the urine. In 14 patients with hepatolenticular degeneration, the total serum Cu values ranged between 35 and 100 ug/100 ml. Ceruloplasmin values were below normal, ranging between 2 and 19 mg/100 ml. In general, there was an increase in the direct-reacting fraction of Cu and a decrease in the oxidase activity. There was little correlation between the total serum Cu values and the ceruloplasmin values. In 5 patients there was good correlation between the indirect-reacting Cu and ceruloplasmin. No correlation was found between the ceruloplasmin level and the duration or severity of the clinical manifestations of the disease, nor were significant quantities of ceruloplasmin found in the urine. In neurologic diseases other than hepatolenticular degeneration the total serum Cu level was either normal or increased and there was no decrease in the quantity of ceruloplasmin in the serum. Ceruloplasmin was found in small quantities in the liver of one "control" subject and in the kidney of 2 "control" subjects. It was not found in brain, spleen, bile, or erythrocytes of the "control" subjects or of the patient with Wilson''s disease. It was found in the liver and kidney of one patient with Wilson''s disease but this protein represented a much smaller proportion of the total Cu demonstrable in these tissues than was the case in the control subjects. The Cu protein in normal erythrocytes was found to be immunologically distinct from ceruloplasmin.