The P blood group system: Biochemical, serological, and clinical aspects

A Single Point Mutation in the Gene Encoding Gb3/CD77 Synthase Causes a Rare Inherited Polyagglutination Syndrome

“…The NOR glycolipids were believed to arise through a mutation in one of the known glycosyltransferases and the expression of an enzyme with altered specificity. The structure that is the most similar to Gal(␣1-4)GalNAc is Gal(␣1-4)Gal, which is present in glycolipids from the P1PK (formerly P) blood group system (8). Serologically, the P1PK blood group system consists of one antigen, P1, whose presence or absence determines the P 1 (P1-positive) or P 2 (P1-negative) blood groups, respectively.…”

Glycosphingolipids in HIV/AIDS: The Potential Therapeutic Application

“…Gb 3 has been implicated in angiogenesis and is found in tumour neovasculature (Heath-Engel & Lingwood, 2003), and can promote tumour metastases (Kovbasnjuk et al, 2005) 3.3 Blood group antigens Gb 3 belongs to the P1PK and GLOB blood group system, that have red cell phenotypes termed P/P 1 /P k (Table 1) and whose expression profile is not limited to erythrocytes. The structure galabiose (Gal1-4Gal) is the terminal structure of P k , also known as Gb 3 , and P 1 blood group antigens, whilst it is the precursor for P antigen, also known as globoside or Gb 4 , which terminates with 1-3GalNAc (Spitalnik & Spitalnik, 1995). P 1 and P 2 are the two common P/P 1 /P k -related blood group phenotypes.…”

“…Individuals who do not express any P/P 1 /P k antigens have mutations in the A4GALT gene, causing lack of functional Gb 3 synthase ( 4Gal transferase), and have the rare p blood group phenotype , Hellberg et al, 2002, Hellberg et al, 2003, Steffensen et al, 2000. Similar to the ABO blood group system, naturally occurring antibodies are formed against the P/P 1 /P k antigens when missing (Spitalnik & Spitalnik, 1995). Recent studies show anti-P k is present in all normal sera (Pochechueva et al, 2010) Phenotype Frequency Red Blood Cell Antigens Serum Antibodies P 1 75% P 1 , P, P k None P 2 25% P, P k Anti-P 1 P 1 k Rare P 1 , P k Anti-P P 2 k Rare P k Anti-P 1, anti-P P Rare None Anti-P 1, anti-P , anti-P k Table 1.…”

“…Recent studies show anti-P k is present in all normal sera (Pochechueva et al, 2010) Phenotype Frequency Red Blood Cell Antigens Serum Antibodies P 1 75% P 1 , P, P k None P 2 25% P, P k Anti-P 1 P 1 k Rare P 1 , P k Anti-P P 2 k Rare P k Anti-P 1, anti-P P Rare None Anti-P 1, anti-P , anti-P k Table 1. Red Blood Cell Phenotypes in the P1Pk and GLOB Blood Group System (Spitalnik & Spitalnik, 1995;Branch, 2010) …”

Molecular Basis of the Globoside-deficient Pk Blood Group Phenotype

“…There are several reasons for the scientific and medical attention spent investigating patients with these rare phenotypes (17). P-related antibodies are implicated in hemolytic transfusion reactions if random antigen-positive blood is transfused.…”