Differences in the terminal steps of complement lysis of normal and paroxysmal nocturnal hemoglobinuria red cells.
The number of microscopically visible lesions produced on the membrane for a given degree of lysis on normal cells as on PNH cells. Since complement lesions were not formed until C8 or C9 was incorporated into the complement sequence, the results suggest that increased lysis of red cells in PNH is due at least in part to more efficient penetration of the PNH membrane by the terminal lytic sequence of complement. Furthermore, the efficiency of the terminal lytic sequence in the lysis of PNH cells when complement was activated by the alternative pathway and the classical pathway was analyzed. There was no significant difference (p less than 0.01) in the number of lesion present at an equivalent degree of lysis when initiated by antibody, cobra venom factor, or acidification. Thus, the efficiency of the terminal lytic sequence does not vary with different modes of activation.
D003185:Complement C8; D003186:Complement C9; D003165:Complement System Proteins; D004910:Erythrocyte Membrane; D004912:Erythrocytes; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood*; D006461:Hemolysis; D006801:Humans
D003185:Complement C8; D003186:Complement C9; D003165:Complement System Proteins