title: Interrelation between structure and protective action of normal and pathological ceruloplasmins during copper-induced lysis of red blood cells.
abstract: 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.
authors.affiliation: A.N.Bach Institute of Biochemistry, USSR Academy of Sciences, Moscow.
authors.forename: E L
authors.initials: EL
authors.lastname: Saenko
authors.forename: O V
authors.initials: OV
authors.lastname: Skorobogat'ko
authors.forename: A I
authors.initials: AI
authors.lastname: Yaropolov
mesh_terms: D002570:Ceruloplasmin / Q000737:chemistry / Q000378:metabolism*; D002853:Chromatography, Liquid; D003300:Copper / Q000097:blood / Q000494:pharmacology*; D004912:Erythrocytes / Q000378:metabolism*; D005978:Glutathione / Q000378:metabolism; D006461:Hemolysis; D006527:Hepatolenticular Degeneration / Q000097:blood*; D006801:Humans; D007700:Kinetics; D011956:Receptors, Cell Surface / Q000378:metabolism; D011971:Receptors, Immunologic; D018000:Receptors, Peptide
chemical_list: D011956:Receptors, Cell Surface; D011971:Receptors, Immunologic; D018000:Receptors, Peptide; C040519:ceruloplasmin receptor; D003300:Copper; D002570:Ceruloplasmin; D005978:Glutathione