Abstract

This chapter discusses the Diego blood group system. The terminology, expression, database accession numbers, and the carrier molecule related to the Diego blood group system are also reviewed. The Diego blood group was named after the producer of the first anti-Dia, discovered during the investigation of a case of HDN in a Venezuelan family. The function and disease association of the Diego blood group system is also discussed. The N-terminus cytoplasmic domain (residues 1–359) is anchored to the membrane skeleton via ankyrin and protein 4.2 and contributes to maintaining the structural integrity of the red blood cell (RBC). The C-terminus membrane domain (residues 360–911) carries out anion exchange (HCO3/Cl) across the RBC membrane and contributes to the stability of the lipid bilayer through interaction with adjacent phospholipid molecules. Band 3 may be involved in the removal of aged or defective RBCs from the circulation (via the formation of senescent antigens) and sequestration of RBCs infected with Plasmodium falciparum.

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