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PMID: 3860874 Published · ppublish English Comparative Study Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, Non-P.H.S. Research Support, U.S. Gov't, P.H.S.

Enhanced complement-mediated lysis of type III paroxysmal nocturnal hemoglobinuria erythrocytes involves increased C9 binding and polymerization.

Proceedings of the National Academy of Sciences of the United States of America ·Vol. 82 ·No. 16 ·1985-08-00 ·Pages 5520-4

Hu VW, Nicholson-Weller A

Abstract

The interaction of terminal complement proteins (C5-C9) with normal erythrocytes and type III paroxysmal nocturnal hemoglobinuria erythrocytes (PNH-E) has been compared in terms of binding of the C5-9 complex, C9 polymerization, and C9 insertion into membranes. Complement components C5, C7, and C8 bind equally well to both types of erythrocytes, whereas the binding of C9 to PNH-E is 5-6 times greater than that to normal erythrocytes. The kinetics of C9 binding was compared with the kinetics of lysis for both types of cells under conditions leading to 100% lysis. There was a noticeable lag time between C9 binding and lysis of normal erythrocytes, but the lysis of PNH-E proceeded without a lag and the kinetics of lysis more closely paralleled C9 binding. The efficiency of C9 insertion was similar for both types of cells, but C9 polymerization was significantly enhanced on PNH-E. These data indicate that the enhanced susceptibility of type III PNH-E toward lysis by C5-9 can be correlated with abnormally high C9 binding and increased formation of poly(C9).

MeSH Terms
Carrier Proteins/immunology,metabolism Complement C9/immunology,metabolism Complement System Proteins/immunology Cytotoxicity, Immunologic Erythrocytes/immunology Hemoglobinuria, Paroxysmal/immunology Hemolysis Humans Kinetics Macromolecular Substances Reference Values
Chemicals
Carrier Proteins Complement C9 Macromolecular Substances Complement System Proteins
Authors & Affiliations
2 authors, click to expand affiliations / ORCID
Hu V W
Nicholson-Weller A
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34 references, click to expand
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Article Info
Journal
Proceedings of the National Academy of Sciences of the United States of America
Abbr.
Proc Natl Acad Sci U S A
ISSN
0027-8424
DOI
10.1073/pnas.82.16.5520
Published
1985-08-00
Pages
5520-4
Language
English
Region
United States
NLM ID
7505876
PMCID
PMC391154
Subset
IM
Grants
NIAID NIH HHS · AI19168 · United States
NHLBI NIH HHS · HL33768 · United States
External Links
PubMed source DOI: 10.1073/pnas.82.16.5520 PMC Full Text View journal details
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