Randall W Velliquette1, Kim Hue-Roye, Christine Lomas-Francis, Barbara Gillen, Jennifer Schierts, Kristie Gentzkow, Thierry Peyrard, Inge von Zabern, Willy A Flegel, Karen Rodberg, Asim K Debnath, Soohee Lee, Marion E Reid. 1. Laboratory of Immunohematology and Genomics, New York Blood Center, Long Island City, New York; Laboratory of Immunochemistry, New York Blood Center, New York, New York; Laboratory of Molecular Modeling and Drug Design, New York Blood Center, New York, New York; Laboratory of Membrane Biochemistry, New York Blood Center, New York, New York; Memorial Blood Centers, St Paul, Minnesota; Medcenter One, Bismarck, North Dakota; National Reference Center for Blood Groups, National Institute of Blood Transfusion, Paris, France; Department of Transfusion Medicine, University Hospital Ulm, Institute of Clinical Transfusion Medicine and Immunogenetics, Ulm and German Red Cross Blood Donor Service, Baden-Württemberg-Hessen, Institute Ulm, Ulm, Germany; Laboratory Services Section, Department of Transfusion Medicine, Clinical Center, National Institutes of Health, Bethesda, Maryland; American Red Cross, Southern California Region, Pomona, California.
Abstract
BACKGROUND: The numerous antigens in the Kell blood group system result from missense nucleotide changes in KEL. Antibodies to antigens in this system can be clinically important. We describe six probands whose plasma contained antibodies to high-prevalence Kell antigens and discuss their relationship. STUDY DESIGN AND METHODS: Polymerase chain reaction amplification, direct sequencing, restriction fragment length polymorphism assays, hemagglutination, flow cytometry, and protein modeling were performed by standard methods. RESULTS: Proband 1 (KUCI) and her serologically compatible sister were heterozygous for a nucleotide change in Exon 11 (KEL*1271C/T; Ala424Val). Proband 2 (KANT) was heterozygous for KEL*1283G/T (Arg428Leu) and KEL*1216C/T (Arg406Stop) in Exon 11. Red blood cells (RBCs) from Proband 1 and her sister were not agglutinated by plasma from Proband 2; however, RBCs from Proband 2 were agglutinated by plasma from Proband 1. Probands 3, 4, 5, and 6 had the KEL*1391C>T change associated with the previously reported KETI- phenotype. Proband 5 was also homozygous for KEL*905T>C encoding the K11-K17+ phenotype. Hemagglutination studies revealed an association between KUCI, KANT, KETI, and K11. Protein modeling indicated that whereas Ala424 and Arg428 are clustered, Val302 and Thr464 are not. CONCLUSION: Ala424 in the Kell glycoprotein is associated with the high-prevalence Kell antigen, KUCI (ISBT 006032), which is detected by the antibody of Proband 1. Arg428 is associated with the high-prevalence Kell antigen, KANT (ISBT 006033). The association between KUCI, KANT, KETI, and K11 and the results of protein modeling are discussed.
BACKGROUND: The numerous antigens in the Kell blood group system result from missense nucleotide changes in KEL. Antibodies to antigens in this system can be clinically important. We describe six probands whose plasma contained antibodies to high-prevalence Kell antigens and discuss their relationship. STUDY DESIGN AND METHODS: Polymerase chain reaction amplification, direct sequencing, restriction fragment length polymorphism assays, hemagglutination, flow cytometry, and protein modeling were performed by standard methods. RESULTS: Proband 1 (KUCI) and her serologically compatible sister were heterozygous for a nucleotide change in Exon 11 (KEL*1271C/T; Ala424Val). Proband 2 (KANT) was heterozygous for KEL*1283G/T (Arg428Leu) and KEL*1216C/T (Arg406Stop) in Exon 11. Red blood cells (RBCs) from Proband 1 and her sister were not agglutinated by plasma from Proband 2; however, RBCs from Proband 2 were agglutinated by plasma from Proband 1. Probands 3, 4, 5, and 6 had the KEL*1391C>T change associated with the previously reported KETI- phenotype. Proband 5 was also homozygous for KEL*905T>C encoding the K11-K17+ phenotype. Hemagglutination studies revealed an association between KUCI, KANT, KETI, and K11. Protein modeling indicated that whereas Ala424 and Arg428 are clustered, Val302 and Thr464 are not. CONCLUSION:Ala424 in the Kell glycoprotein is associated with the high-prevalence Kell antigen, KUCI (ISBT 006032), which is detected by the antibody of Proband 1. Arg428 is associated with the high-prevalence Kell antigen, KANT (ISBT 006033). The association between KUCI, KANT, KETI, and K11 and the results of protein modeling are discussed.
Authors: S Lee; D C Russo; A P Reiner; J H Lee; M Y Sy; M J Telen; W J Judd; P Simon; M J Rodrigues; T Chabert; J Poole; S Jovanovic-Srzentic; C Levene; V Yahalom; C M Redman Journal: J Biol Chem Date: 2001-05-24 Impact factor: 5.157
Authors: Narayanan Eswar; Bino John; Nebojsa Mirkovic; Andras Fiser; Valentin A Ilyin; Ursula Pieper; Ashley C Stuart; Marc A Marti-Renom; M S Madhusudhan; Bozidar Yerkovich; Andrej Sali Journal: Nucleic Acids Res Date: 2003-07-01 Impact factor: 16.971
Authors: G Daniels; L Castilho; W A Flegel; A Fletcher; G Garratty; C Levene; C Lomas-Francis; J M Moulds; J J Moulds; M L Olsson; M Overbeeke; J Poole; M E Reid; P Rouger; E van der Schoot; M Scott; P Sistonen; E Smart; J R Storry; Y Tani; L-C Yu; S Wendel; C Westhoff; V Yahalom; T Zelinski Journal: Vox Sang Date: 2009-02 Impact factor: 2.144
Authors: Ursula Pieper; Benjamin M Webb; David T Barkan; Dina Schneidman-Duhovny; Avner Schlessinger; Hannes Braberg; Zheng Yang; Elaine C Meng; Eric F Pettersen; Conrad C Huang; Ruchira S Datta; Parthasarathy Sampathkumar; Mallur S Madhusudhan; Kimmen Sjölander; Thomas E Ferrin; Stephen K Burley; Andrej Sali Journal: Nucleic Acids Res Date: 2010-11-19 Impact factor: 16.971
Authors: Edmir Boturão-Neto; Mihoko Yamamoto; Akemi Kuroda Chiba; Elisa Yuriko Sugano Kimura; Maria do Carmo Valgueiro Costa de Oliveira; Cláudia Lumack do Monte Barretto; Mércia Maria Alves Nunes; Sérgio Roberto Lopes Albuquerque; Marcos Daniel de Deus Santos; José Orlando Bordin Journal: Transfus Med Hemother Date: 2014-12-19 Impact factor: 3.747