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. 1972 Sep;46(1):157–166. doi: 10.1111/j.1476-5381.1972.tb06858.x

Activation of arginine and tyrosine esterase in serum from patients with hereditary angio-oedema

V Eisen, C Loveday
PMCID: PMC1666111  PMID: 5084818

Abstract

1. The role of clotting factor XII in the activation of the complement subunit C1s to C1 esterase was examined.

2. In sera from patients with hereditary angio-oedema who lack the α2-glycoglobulin C1 inhibitor, silicates and other potent activators of clotting factor XII induced far less C1 esterase activity than did the weaker factor XII activators, carrageenin and cellulose sulphate. In contrast, the intensity of the induced plasma kallikrein activity corresponded more closely to the clot-promoting effect of the factor XII activators.

3. Spontaneous generation of C1 esterase activity was only slightly delayed in hereditary angio-oedema sera previously depleted of factor XII. In normal sera, C1 esterase did not develop spontaneously and could not be induced.

4. Experiments with inhibitors suggested that the spontaneous activation of C1s may consist of two phases: factor XII and other plasma proteases first activate small amounts of C1s; the resulting C1 esterase then activates the bulk of C1s. The observed spontaneous activation suggests that when fully activated, the C1s present in 1 ml of human serum will hydrolyse 1-2 μmol of ATEe/minute.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. DONALDSON V. H., EVANS R. R. A BIOCHEMICAL ABNORMALITY IN HEREDIATRY ANGIONEUROTIC EDEMA: ABSENCE OF SERUM INHIBITOR OF C' 1-ESTERASE. Am J Med. 1963 Jul;35:37–44. doi: 10.1016/0002-9343(63)90162-1. [DOI] [PubMed] [Google Scholar]
  2. Donaldson V. H. Mechanisms of activation of C'1 esterase in hereditary angioneurotic edema plasma in vitro. J Exp Med. 1968 Mar 1;127(3):411–429. doi: 10.1084/jem.127.3.411. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Donaldson V. H., Rosen F. S. Hereditary angioneurotic edema: a clinical survey. Pediatrics. 1966 Jun;37(6):1017–1027. [PubMed] [Google Scholar]
  4. EISEN V. EFFECT OF HEXADIMETHRINE BROMIDE ON PLASMA KININ FORMATION, HYDROLYSIS OF P-TOSYL-L-ARGININE METHYL ESTER AND FIBRINOLYSIS. Br J Pharmacol Chemother. 1964 Feb;22:87–103. doi: 10.1111/j.1476-5381.1964.tb01546.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Forbes C. D., Pensky J., Ratnoff O. D. Inhibition of activated Hageman factor and activated plasma thromboplastin antecedent by purified serum C1 inactivator. J Lab Clin Med. 1970 Nov;76(5):809–815. [PubMed] [Google Scholar]
  6. Hadjiyannaki K., Lachmann P. J. Hereditary angio-oedema: a review with particular reference to pathogenesis and treatment. Clin Allergy. 1971 Jun;1(2):221–233. doi: 10.1111/j.1365-2222.1971.tb03021.x. [DOI] [PubMed] [Google Scholar]
  7. Kaplan A. P., Austen K. F. A pre-albumin activator of prekallikrein. J Immunol. 1970 Oct;105(4):802–811. [PubMed] [Google Scholar]
  8. Kellermeyer W. F., Jr, Kellermeyer R. W. Hageman factor activation and kinin formation in human plasma induced by cellulose sulfate solutions. Proc Soc Exp Biol Med. 1969 Apr;130(4):1310–1314. doi: 10.3181/00379727-130-33780. [DOI] [PubMed] [Google Scholar]
  9. LANDERMAN N. S., WEBSTER M. E., BECKER E. L., RATCLIFFE H. E. Hereditary angioneurotic edema. II. Deficiency of inhibitor for serum globulin permeability factor and/or plasma kallikrein. J Allergy. 1962 Jul-Aug;33:330–341. doi: 10.1016/0021-8707(62)90032-1. [DOI] [PubMed] [Google Scholar]
  10. LEVY L. R., LEPOW I. H. Assay and properties of serum inhibitor of C'l-esterase. Proc Soc Exp Biol Med. 1959 Aug-Sep;101:608–611. doi: 10.3181/00379727-101-25034. [DOI] [PubMed] [Google Scholar]
  11. Lachmann P. J. E. Complement. The immunochemistry of complement. Proc R Soc Lond B Biol Sci. 1969 Jul 1;173(1032):371–376. doi: 10.1098/rspb.1969.0063. [DOI] [PubMed] [Google Scholar]
  12. Lundh B., Laurell A. B., Wetterqvist H., White T., Granerus G. A case of hereditary angioneurotic oedema, successfully treated with epsilon-aminocaproic acid. Studies on C'1 esterase inhibitor, C'1 activation, plasminogen level and histamine metabolism. Clin Exp Immunol. 1968 Sep;3(7):733–745. [PMC free article] [PubMed] [Google Scholar]
  13. MARGOLIS J. Activation of plasma by contact with glass: evidence for a common reaction which releases plasma kinin and initiates coagulation. J Physiol. 1958 Nov 10;144(1):1–22. doi: 10.1113/jphysiol.1958.sp006082. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. MARGOLIS J. The kaolin clotting time; a rapid one-stage method for diagnosis of coagulation defects. J Clin Pathol. 1958 Sep;11(5):406–409. doi: 10.1136/jcp.11.5.406. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Movat H. Z., Poon M. C., Takeuchi Y. The kinin-system of human plasma. I. Isolation of a low molecular weight activator of prekallikrein. Int Arch Allergy Appl Immunol. 1971;40(1):89–112. doi: 10.1159/000230397. [DOI] [PubMed] [Google Scholar]
  16. Müller-Eberhard H. J. Complement. Annu Rev Biochem. 1969;38:389–414. doi: 10.1146/annurev.bi.38.070169.002133. [DOI] [PubMed] [Google Scholar]
  17. Naff G. B., Ratnoff O. S. The enzymatic nature of C'1r. Conversion of C'1s to C'1 esterase and digestion of amino acid esters by C'1r. J Exp Med. 1968 Oct 1;128(4):571–593. doi: 10.1084/jem.128.4.571. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. RATNOFF O. D., MILES A. A. THE INDUCTION OF PERMEABILITY-INCREASING ACTIVITY IN HUMAN PLASMA BY ACTIVATED HAGEMAN FACTOR. Br J Exp Pathol. 1964 Jun;45:328–345. [PMC free article] [PubMed] [Google Scholar]
  19. Ratnoff O. D., Naff G. B. The conversion of C'IS to C'1 esterase by plasmin and trypsin. J Exp Med. 1967 Feb 1;125(2):337–358. doi: 10.1084/jem.125.2.337. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Ratnoff O. D., Pensky J., Ogston D., Naff G. B. The inhibition of plasmin, plasma kallikrein, plasma permeability factor, and the C'1r subcomponent of the first component of complement by serum C'1 esterase inhibitor. J Exp Med. 1969 Feb 1;129(2):315–331. doi: 10.1084/jem.129.2.315. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Schur P. H., Austen K. F. Complement in human disease. Annu Rev Med. 1968;19:1–24. doi: 10.1146/annurev.me.19.020168.000245. [DOI] [PubMed] [Google Scholar]
  22. van OSS C., FRIEDMANN J. C., FONTAINE M. Anticomplementary action of aspirin. Nature. 1961 Jan 14;189:147–147. doi: 10.1038/189147a0. [DOI] [PubMed] [Google Scholar]

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