SUMMARY. Human red cells incubated with a solution containing 6% 2-aminoethylisothiouronium bromide (AET) lose activity of antigens that are part of, or related to, the Kell blood group system. However, Kx antigen is not inactivated. Studies on a wide range of other blood-group antigens show no other evidence of changes and AET appears to react specifically with red-cell membrane structures that have Kell activity. The AET procedure produces an artificial KO red cell that can be used in blood group serology, and allows easy recognition of antibodies that are associated with the Kell system. AET has been used by other workers to produce a red cell that has many serological and biochemical characteristics of a PNH cell. Our studies on red cells from PNH patients have not shown any changes in Kell blood-group antigens.

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Inactivation of Kell blood group antigens by 2-aminoethylisothiouronium bromide

Oxidative stress in paroxysmal nocturnal hemoglobinuria and other conditions of complement-mediated hemolysis.

The mammalian erythrocyte has occupied a position at the forefront of enzymology since the early days of physiological chemistry. Indeed, the erythrocyte continues to occupy a place of prominence, with respect to the enzymology of both its membranous and cytosolic systems (Beutler, 1975, 1986; Brewer, 1984; Friedman and Rapoport, 1974; Schrier, 1977).

Some High-Molecular-Weight Oligomeric Proteins and Enzymes of Reticulocytes and Erythrocytes

Functional study of lipids of PNH red cell membranes: susceptibility of liposomes to reactive lysis

Publisher Summary Paroxysmal nocturnal hemoglobinuria (PNH) is a disease of bone marrow stem cells. This disease is also known as the Marchiafava–Micheli syndrome. No effective therapy, however, has been developed to date. The mechanisms accounting for the venous thrombosis that is often the fatal manifestation are still unknown. The elucidation of glycosylphosphatidylinositol (GPI)-anchor biosynthesis as well as the characterization of other functional GPI-anchored proteins would be essential for the development of a therapy for this disease. A few patients with PNH show a lessening of the severity of the clinical features and incidentally show a complete remission of PNH, indicating that the affected stem cells can, under certain circumstances, lose their advantage for proliferation in bone marrow. The elucidation of this apparently spontaneous remission would provide a fundamental therapy for PNH.

Chapter 10 Paroxysmal nocturnal hemoglobinuria

Proteins obtained from membranes of PNH erythrocytes and of red cells treated with 2-aminoethylisothiouronium bromide (AET) following lysis in acidified serum (Ham's test) were compared by sodium dodecylsulphate-polyacrylamide gel electrophoresis. The peptide patterns were identical, providing support that AET-treated red cells afford a suitable experimental substitute for PNH red cells in studies involving complement fixation.

Noppaporn Burapakulsolsri

Papers

An examination of complement proteins on membranes of paroxysmal nocturnal haemoglobinuria (PNH) and PNH-like red cells.