Interrelation between structure and protective action of normal and pathological ceruloplasmins during copper-induced...

The origin of the difference between the protective action of ceruloplasmin (CP) from healthy donors blood and of ceruloplasmin-like protein (p-CP) from blood of patients with Wilson disease which they exert during copper-induced lysis of red blood cells (RBC) was elucidated. The difference is due to a significant change in the carbohydrate moiety of p-CP the major proportion of which (65%) does not contain mannose and acetylglucosamine residues. The data of chromatography on lentil lectin reveal that only 4% of p-CP molecules contain the fragment [table: see text] required for binding to RBC receptors. It was shown that the time-courses of copper accumulation in RBC of normal donors and in RBC of patients with Wilson disease (p-RBC) during copper-induced lysis differ markedly from each other. The p-CP is able to prevent copper accumulation in RBC and p-RBC to a significantly less degree than CP. It was also established that CP prevents the decrease of reduced glutathione (GSH) level in RBC to a greater extent than p-CP. In contrast to CP, the p-CP exerts no effect on the decrease in GSH concentration in p-RBC. These results may indicate that no interaction between Cu2+ and reduced glutathione takes place in p-RBC, in contrast to the situation occurring in normal RBC.