Figure 4.
Demonstration that paroxysmal nocturnal hemoglobinuria (PNH) erythrocytes are deficient in MIRL (CD59), the glycosyl phosphatidylinositol (GPI)–anchored protein that inhibits formation of the membrane attack complex of complement. Illustrated is analysis by western blot of normal (NL) and PNH erythrocyte membrane proteins using anti-MIRL as the primary antibody. A band representing a protein with a molecular mass of ~18 kilodaltons is present in the lanes containing normal erythrocyte membrane proteins (NL1 and NL2). A band with this mass is absent in the lane containing the PNH erythrocytes membrane proteins (PNH). Deficiency of MIRL (CD59) and DAF (CD55) accounts for the abnormal sensitivity of PNH erythrocytes to complement-mediated lysis.
Reproduced from
Holguin MH, Fredrick LR, Bernshaw NJ, Wilcox LA, Parker CJ. Isolation and characterization of a membrane protein from normal human erythrocytes that inhibits reactive lysis of the erythrocytes of paroxysmal nocturnal hemoglobinuria. J Clin Invest. 1989;84:7–17
by copyright permission of The American Society of Clinical Investigation.