PMID: 6019133Feb 1, 1967Paper

Formation and functional significance of a molecular complex derived from the second and the fourth component of human complement

The Journal of Experimental Medicine
H J Müller-EberhardM A Calcott

Abstract

A functional unit of the human complement system has been delineated. It is composed of two subunits which are derived from the second (C'2) and from the fourth (C'4) component of complement. Purified C'2 and C'4 were found to interact in free solution and to form a reversible protein-protein complex. When acted upon by C'1 esterase in the presence of Mg ions, the reversible complex acquires stability and the ability to act enzymatically on the third component of complement. The trivial name C'3 convertase has been selected to denote the enzyme. Molecular weight determinations suggest that the entire C'4 molecule, but probably only a fragment of the C'2 molecule, are incorporated into C'3 convertase. Prior treatment of C'2 with iodine led to enhanced stability and activity of the enzyme. It was shown that the cell-bound form of C'3 convertase is cytolytically active and that the free enzyme is able to induce lysis from the fluid phase of erythrocytes from patients with paroxysmal nocturnal hemoglobinuria. Evidence was presented that action of C'3 convertase on C'3 results in fragmentation of the molecule.

References

Jan 1, 1966·The Journal of Experimental Medicine·H J MüllerèberhardM A Calcott
Jan 1, 1966·International Archives of Allergy and Applied Immunology·P J McConahey, F J Dixon
May 1, 1966·Immunochemistry·R M StroudA T McKenzie
Sep 1, 1963·The Journal of Experimental Medicine·H J MUELLER-EBERHARD, C E BIRO
Apr 1, 1964·The Journal of Experimental Medicine·G B NAFFI H LEPOW
May 1, 1965·The Journal of Experimental Medicine·H J MUELLER-EBERHARD, I H LEPOW

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Citations

Feb 1, 1997·Protein Science : a Publication of the Protein Society·S K Law, A W Dodds
Jan 1, 1984·Springer Seminars in Immunopathology·H J Müller-Eberhard
Aug 1, 1983·Agents and Actions·W Vogt
May 1, 1975·Immunochemistry·J L MolenaarK W Pondman
Jul 16, 1985·Journal of Immunological Methods·A Lundwall, G Eggertsen
Dec 1, 1982·Molecular Immunology·A LundwallJ Sjöquist
May 1, 1988·Molecular Immunology·M Kirschfink, T Borsos
May 15, 2010·Nature Reviews. Microbiology·Davide SerrutoJos A G van Strijp
Sep 7, 1972·The New England Journal of Medicine·S RuddyK F Austen
Mar 22, 1973·The New England Journal of Medicine·C A AlperF S Rosen
Apr 19, 1973·The New England Journal of Medicine·R B JohnstonA G Struth
Oct 1, 1972·Proceedings of the National Academy of Sciences of the United States of America·M M Mayer
Aug 1, 1975·Proceedings of the National Academy of Sciences of the United States of America·D T Fearon, K F Austen
Feb 1, 1976·Proceedings of the National Academy of Sciences of the United States of America·R G MedicusH J Müller-Eberhard
Sep 1, 1976·Proceedings of the National Academy of Sciences of the United States of America·J M WeilerD T Fearon
Apr 1, 1977·Proceedings of the National Academy of Sciences of the United States of America·D T Fearon, K F Austen
Jul 1, 1977·Proceedings of the National Academy of Sciences of the United States of America·S Nagasawa, R M Stroud
Dec 1, 1979·Proceedings of the National Academy of Sciences of the United States of America·I GigliV Nussenzweig
Oct 1, 1980·Proceedings of the National Academy of Sciences of the United States of America·B F TackJ W Prahl
May 1, 1982·Proceedings of the National Academy of Sciences of the United States of America·P VanguriM L Shin
Aug 1, 1983·Proceedings of the National Academy of Sciences of the United States of America·A Nicholson-WellerK F Austen
Dec 1, 1967·The Journal of Experimental Medicine·M J Polley, H J Müller-Eberhard
Feb 1, 1968·The Journal of Experimental Medicine·C G Cochrane, H J Müller-Eberhard
Jun 1, 1969·The Journal of Experimental Medicine·R B JohnstonF S Rosen
Feb 1, 1973·The Journal of Experimental Medicine·N R Cooper
Aug 1, 1973·The Journal of Experimental Medicine·W P KolbH J Müller-Eberhard
Aug 1, 1974·The Journal of Experimental Medicine·D T FearonS Ruddy
Nov 1, 1974·The Journal of Experimental Medicine·R D Schreiber, H J Müller-Eberhard
Oct 1, 1977·The Journal of Experimental Medicine·A FerreiraV Nussenzweig
Mar 1, 1981·The Journal of Experimental Medicine·B GhebrehiwetA P Kaplan

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