NOR polyagglutination and St(a) glycophorin in one family: relation of NORpolyagglutination to terminal alpha-galactose residues and abnormal glycolipids

BACKGROUND: This report describes the characterization of polyagglutinable red cells (RBCs), identified in two generations of a Polish family. CASE REPORT: Untreated and modified RBCs of the proposita (TS) were tested by serologic methods, using human sera, antibodies, lectins, and inhibitors of agglutination. Moreover glycophorins were characterized by sodium docec yl sulfate-polyacrylamide gel electrophoresis and Western blotting, and gly colipids were purified, fractionated by thin-layer chromatography, and dete cted with Ricinus communis agglutinin I (RCA-I, specific for galactose resi dues) and Griffonia simplicifolia IB4 lectin (GSL-IB4, specific for Gal alp ha 1-3Gal- structure). Some of the experiments were also performed on RBCs of members of TS's family. RESULTS: Polyagglutination, found in four members of TS's family, was ident ified as the second case of an earlier described NOR polyagglutination. The polyagglutination was decreased by treating the RBCs with a-galactosidase and was inhibited by a neutral glycolipid fraction from NOR+ RBCs. Detectio n of neutral glycolipids of TS's RBCs on the thin-layer plate by RCA-I and GSL-IB4 revealed the presence of components that were not detectable in con trol RBCs. Moreover, Western blotting of RBC membranes from five family mem bers with glycophorin monoclonal antibodies and agglutination assays with a nti-St(a) and anti-Dantu sera identified the presence of St(a) glycophorin in four members of the family, two of whom were NOR+ and two NOR-. CONCLUSION: Our results showed that two rare features of TS's RBCs, NOR pol yagglutination and St(a) glycophorin, are inherited independently, and that NOR+ RBCs contain neutral glycolipids with an abnormal oligosaccharide str ucture, most likely terminated with alpha-galactosyl residues.