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.
Department of Biochemistry, Midnapore Medical College, Paschim Medinipur, WB 721101 India.
Nabarun
N
Mandal
Department of Biochemistry, Murshidabad Medical College, Berhampore, Murshidabad, WB 703482 India.
Debojyoti
D
Bhattacharjee
Department of Biochemistry, R.G.Kar Medical College, Kolkata, WB 700004 India.
Jayanta Kumar
JK
Rout
Department of Biochemistry, Calcutta National Medical College, Kolkata, WB 700014 India.
Anindya
A
Dasgupta
Department of Biochemistry, MGM Medical College, Kishanganj, Bihar 855107 India.
Gorachand
G
Bhattacharya
Department of Biochemistry, R.G.Kar Medical College, Kolkata, WB 700004 India.
Chandan
C
Sarkar
Department of Neurology, KPC Medical College, Kolkata, WB 700032 India.
Prasanta Kumar
PK
Gangopadhyaya
Human erythrocyte; Reduced glutathione; Wilson’s disease; l-Cysteine/l-cystine influx