Differences in the terminal steps of complement lysis of normal and paroxysmal nocturnal hemoglobinuria red cells

Author:

Rouault TA,Rosse WF,Bell S,Shelburne J

Abstract

Abstract 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.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Cited by 20 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. Complement and PNH;Paroxysmal Nocturnal Hemoglobinuria;2017

2. Historical aspects of paroxysmal nocturnal haemoglobinuria: ‘defining the disease’;British Journal of Haematology;2002-03-25

3. Hemolysis in PNH;PNH and the GPI-Linked Proteins;2000

4. Pathogenesis and management of paroxysmal nocturnal haemoglobinuria;Blood Reviews;1998-03

5. Autoimmune Hemolytic Anemia;The Human Complement System in Health and Disease;1998-02-25

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