This website was created by Fumiichiro Yamamoto, Ph.D., to disseminate knowledge on the molecular genetic basis of the ABO system.

Karl Landsteiner discovered the ABO system in 1900, distinguishing it as one of the most important blood group systems in transfusion medicine. The system consists of A and B antigens and their corresponding antibodies. The underlying factor differentiating the ABO system from others, such as the Rh system, is the presence of antibodies against A and B antigens. These antibodies are present in individuals who do not express A and B antigens, and cause the first mismatched blood transfusion to be possibly fatal. The discovery of the ABO blood group system paved the way for safe blood transfusion.

Due to its complexity, exploration of the ABO system peaks interest not only in transfusion medicine, but also in a variety of scientific fields. In addition to the four major groups (A, B, AB, O), we know of more than a dozen existing subgroups that exhibit different patterns and degrees of agglutination. Additionally, A and B antigens are found not only on red blood cells (RBCs) but also on the surface of other cell types and in secretions. As such, the system is often referred to as the “histo-blood group system.” The presence of A and B antigens on cells other than RBCs emphasizes the importance of ABO blood type matching not only in blood transfusions, but also in cell, tissue, and organ transplantations.

Both the synthesis and properties of A and B antigens raises many important questions on their roles not only in medicine but also in many aspects of biology. A and B antigens are synthesized by a series of enzymatic reactions catalyzed by enzymes called glycosyltransferases. In fact, the final step in producing these antigens requires a glycosyltransferase, which is encoded by the functional A and B alleles at the ABO genetic locus. The fact that allele frequencies vary amongst different races raises interesting questions on the relevance of ABO blood type on population studies, anthropology and human genetics. Another interesting characteristic of A and B antigens is their presence in animals other than human beings. The glycosyltransferases involved in A/B antigen production in humans also exhibit the same enzymatic purposes in animals. Therefore, the ABO blood group system is also of evolutionary and enzymatic significance. A/B antigens also exhibit dynamic changes during development and pathogenesis, suggesting their importance in cancer, molecular, cellular and developmental biology.

Safer blood transfusion, conceived by Landsteiner and improved upon by many others, primarily immunohematologists, has become a routine medical practice. Since our cloning of the ABO gene in 1990, progress has been made in the structural and functional analyses of ABO genes and A/B transferases at the molecular level. I hope that readers find these web pages interesting and useful, and that they both help facilitate a better understanding of the scientific bases of the ABO system, oligosaccharide ABH antigens, A and B transferases, and ABO genes, and aid in applying this information to clinical applications.

02. Immunogenetics of the Histo-blood group ABO System

03. Discovery of ABO Blood Group System

04. A and B Antigens

05. A and B transferases

06. A transferase cDNA cloning

07. Northern Hybridization Results

08. A/B/O Allelic cDNAs

09. Deduced Amino Acid Sequences of A/B/O Alleles

10. Restriction Fragment Length Polymorphism (A/B Allele vs. O Allele)

11. RFLP (A/O Alleles vs. B Allele)

12. ABO Genotyping of Blood Specimens

13. ABO Alleles (A and B Alleles)

14. ABO Alleles (O Alleles)

15. ABO Alleles (A and B Subgroup Alleles)

16. ABO Alleles (cis-AB & B(A) Alleles)

17. ABO Alleles 2008

18. ABO Alleles (BGAGMD)

19. ABO Allele Mutations

20. Transfection Analysis

21. Transfection Results (A/B transferase)

22. Transfection Results (A2 and A3 Alleles)

23. Transfection Results (O Alleles)

24. Transfection Results (A-B Transferase Chimeras --AA)

25. Transfection Results (A-B Transferase Chimeras --BB)

26. Transfection Results (A-B Transferase Chimeras --AB)

27. Transfection Results (A-B Transferase Chimeras --BA)

28. Transfection Results (A transferase Codon 268)

29. Nucleotide-Sugar Specificity (A transferase Codons 266-268)

30. Transfection Results (B transferase Codon 268)

31. Nucleotide-Sugar Specificity (B Transferase Codons 266-268)

32. Three-Dimensional Structure of A Transferase

33.Homologous Sequences in Other Species

34. Primate ABO Gene Sequence Comparison

35. Evolution of ABO Genes in Primates

36. Evolution of ABO Genes (2008)

37. Alpha 1-3 Gal(NAc) Transferase Family

38. Evolutionary Tree of ABO and Related Genes (2001)

39. Partial Amino Acid Sequence Comparison (2001)

40. Evolutionary Tree of ABO and Related Genes (2008)

41. Partial Amino Acid Sequence Comparison (2008)

42. Partial Amino Acid Sequence Comparison (GGTA1 Genes)

43. Partial Amino Acid Sequence Comparison (A3GALT2 Genes)

44. Partial Amino Acid Sequence Comparison (GBGT1 Genes)

45. Partial Amino Acid Sequence Comparison (ABO Genes)

46. Partial Amino Acid Sequence Comparison (GLT6D1 Genes)

47. Glycosyltransferase Gene Family

48. Glycosyltransferase Gene Expression in Human Tissues

49. Glycosyltransferase Gene Expression Analyzed by Hierarchical Clustering Algorithm

50. Summary

51. Acknowledgment

52. Yamamoto Biosketch 

Sitemap

Appendix 01.Discovery of the ABO blood group system 

Appendix 02. Four major groups 

Appendix 03. Genetic basis of ABO blood grouping 

Appendix 04. ABH(O) substances 

Appendix 05. Immuno-determinant structures of ABH(O) antigens 

Appendix 06. The immuno-dominant sugars of the A and B antigens are GalNAc (N-acetyl-D-galactosamine 

Appendix 07. The Biosynthetic pathways of the A and B antigens 

Appendix 08. Linkage analysis of the ABO genes 

Appendix 09. ABO blood groups and A and B transferase activity 

Appendix 10. History of the purification attempt of A transferase 

Appendix 11. A and B subgroups 

Appendix 12. A1 and A2 subgroups 

Appendix 13. A3, Ax, and B3 weak subgroups 

Appendix 14. Discovery of cis-AB 

Appendix 15. Two examples of cis-AB inheritance 

Appendix 16. Discovery of B(A) phenotype 

Appendix 17. Mapping of the ABO gene using the radiation hybrid panel 

Appendix 18. ABH and related antigens 

Appendix 19. The genomic structure of the human ABO gene 

Appendix 20. Comparison of amino acid sequences of the ABO and related genes 

Appendix 21. Comparison of gene organization between human and mouse ABO genes 

Appendix 22. Polymorphism in the ABO gene that was observed among different species and subspecies o

Appendix 23. The specificity of the murine enzyme 

Appendix 24. Porcine ABO gene 

Appendix 25. A variety of methods for the ABO genotyping