Abstract
The P1PK blood group system (ISBT no. 003) was previously called the P blood group system, and the number of included antigens has varied over time. The history of the system is complicated and sometimes confusing, since several changes to the nomenclature have been made. Furthermore, the association between the antigens and their genetic home has raised many questions, as well as the long‐standing enigma regarding the molecular mechanism underlying the common P1 and P2 phenotypes. Step by step, the biochemical and genetic basis underlying the antigens expressed in this system has been revealed. This review will start with the historical background and continue with the latest findings, answering some of the questions such as why individuals with p phenotype lack not only Pk and P expression, but also P1, and whether the P1 antigens exist on both glycolipids and glycoproteins on the human red blood cells.
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