Effect of Copper on l -Cysteine/ l -Cystine Influx in Normal Human Erythrocytes and Erythrocytes of Wilson’s Disease

Wilson’s disease is a disease of abnormal copper metabolism in which free serum copper level is raised. The objective of the study was to determine, whether in Wilson disease, l -cysteine/ l -cystine influx into RBC was decreased or not and the specific amino acid transporter affected by copper in normal human RBC. For l -cysteine/ l -cystine influx, ten untreated cases, ten treated cases and ten age and sex matched healthy controls were recruited. To study the effect of copper on l -cysteine/ l -cystine influx in RBC, 15 healthy subjects were selected. RBC GSH and l -cysteine/ l -cystine influx were estimated by Beautler’s and Yildiz’s method respectively. In untreated cases, l -cysteine/ l -cystine influx and erythrocyte GSH level were decreased showing that elevated level of free copper in serum or media decreased l -cysteine/ l -cystine influx in human RBC. Copper treatment inhibited L amino acid transporter in normal RBC specifically.