Mechanisms of Activation of C'1 Esterase in Hereditary Angioneurotic Edema Plasma in Vitro

Overview

Journal J Exp Med

Specialties Allergy & Immunology
General Medicine

Date 1968 Mar 1

PMID 5299945

Citations 27

Authors

V H DONALDSON

Affiliations

Soon will be listed here.

Abstract

The generation of C'1 esterase activity in siliconed plasma obtained from individuals with hereditary angioneurotic edema in remission tends to occur spontaneously, but can be hastened during its incubation with preparations of activated Hageman factor. This effect of activated Hageman factor could not be shown during its incubation with normal siliconed plasma, nor could consumption of normal serum inhibition of C'1 esterase be clearly shown. Soy bean trypsin inhibitor and heparin could impair this enhanced generation of C'1 esterase but neither inhibits the esterolytic function of C'1 esterase once formed. Trasylol was less effective in blocking this effect of activated Hageman factor. While the mechanism of the effect of activated Hageman factor upon C'1 activation remains obscure, it is apparent that some intermediate steps, possibly involving a kinin-forming system of plasma, may play a role.

Citing Articles

Complement, Kinins, and Hereditary Angioedema: Mechanisms of Plasma Instability when C1 Inhibitor is Absent.

Kaplan A, Joseph K Clin Rev Allergy Immunol. 2016; 51(2):207-15.

PMID: 27273087 DOI: 10.1007/s12016-016-8555-6.

Bradykinin and the pathogenesis of hereditary angioedema.

Kaplan A World Allergy Organ J. 2013; 4(4):73-5.

PMID: 23282402 PMC: 3651110. DOI: 10.1097/WOX.0b013e318216b7b2.

The inheritance pattern of factor XII (Hageman) deficiency in domestic cats.

Kier A, Bresnahan J, White F, Wagner J Can J Comp Med. 1980; 44(3):309-14.

PMID: 7427778 PMC: 1320079.

Activation of the classical pathway of complement by Hageman factor fragment.

Ghebrehiwet B, Silverberg M, Kaplan A J Exp Med. 1981; 153(3):665-76.

PMID: 7252410 PMC: 2186101. DOI: 10.1084/jem.153.3.665.

Inactivation of factor XII active fragment in normal plasma. Predominant role of C-1-inhibitor.

De Agostini A, Lijnen H, Pixley R, Colman R, Schapira M J Clin Invest. 1984; 73(6):1542-9.

PMID: 6725552 PMC: 437064. DOI: 10.1172/JCI111360.

References

Davie E, Ratnoff O . WATERFALL SEQUENCE FOR INTRINSIC BLOOD CLOTTING. Science. 1964; 145(3638):1310-2. DOI: 10.1126/science.145.3638.1310. View

Ratnoff O, Rosenblum J . Role of Hageman factor in the initiation of clotting by glass; evidence that glass frees Hageman factor from inhibition. Am J Med. 1958; 25(2):160-8. DOI: 10.1016/0002-9343(58)90023-8. View

Ratnoff O, Naff G . The conversion of C'IS to C'1 esterase by plasmin and trypsin. J Exp Med. 1967; 125(2):337-58. PMC: 2138350. DOI: 10.1084/jem.125.2.337. View

WEBSTER M, PIERCE J . Action of the kallikreins on synthetic ester substrates. Proc Soc Exp Biol Med. 1961; 107:186-91. DOI: 10.3181/00379727-107-26575. View

Ratnoff O, Davie E . The purification of activated Hageman factor (activated factor XII). Biochemistry. 1962; 1:967-75. DOI: 10.1021/bi00912a005. View

Margolis J . Hagerman factor and capillary permeability. Aust J Exp Biol Med Sci. 1959; 37:239-44. DOI: 10.1038/icb.1959.24. View

DONALDSON V, Rosen F . ACTION OF COMPLEMENT IN HEREDITARY ANGIONEUROTIC EDEMA: THE ROLE OF C'1-ESTERASE. J Clin Invest. 1964; 43:2204-13. PMC: 442008. DOI: 10.1172/JCI105094. View

MIRSKY I . INHIBITION OF beta HEMOLYTIC STREPTOCOCCI FIBRINOLYSIN BY TRYPSIN INHIBITOR (ANTIPROTEASE). Science. 1944; 100(2592):198-200. DOI: 10.1126/science.100.2592.198. View

Koch W . Relationship between air temperature and mean radiant temperature in thermal comfort. Nature. 1962; 196:587. DOI: 10.1038/196587a0. View

10.

Ratnoff O, CRUM J . ACTIVATION OF HAGEMAN FACTOR BY SOLUTIONS OF ELLAGIC ACID. J Lab Clin Med. 1964; 63:359-77. View

11.

BURDON K, Queng J, Thomas O, McGOVERN J . Observations on biochemical abnormalities in hereditary angioneurotic edema. J Allergy. 1965; 36(6):546-57. DOI: 10.1016/0021-8707(65)90192-9. View

12.

Ratnoff O, Conley C . The role of surface and of calcium in the coagulation of a globulin fraction of platelet-deficient plasma. Bull Johns Hopkins Hosp. 1951; 89(3):245-54. View

13.

MACFARLANE R . AN ENZYME CASCADE IN THE BLOOD CLOTTING MECHANISM, AND ITS FUNCTION AS A BIOCHEMICAL AMPLIFIER. Nature. 1964; 202:498-9. DOI: 10.1038/202498a0. View

14.

LANDERMAN N, WEBSTER M, Becker E, RATCLIFFE H . Hereditary angioneurotic edema. II. Deficiency of inhibitor for serum globulin permeability factor and/or plasma kallikrein. J Allergy. 1962; 33:330-41. DOI: 10.1016/0021-8707(62)90032-1. View

15.

Kagen L . SOME BIOCHEMICAL AND PHYSICAL PROPERTIES OF THE HUMAN PERMEABILITY GLOBULINS. Br J Exp Pathol. 1964; 45:604-11. PMC: 2093675. View

16.

ALKJAERSIG N, Fletcher A, Sherry S . xi-Aminocaproic acid: an inhibitor of plasminogen activation. J Biol Chem. 1959; 234(4):832-7. View

17.

Pensky J, LEVY L, Lepow I . Partial purification of a serum inhibitor of C'1-esterase. J Biol Chem. 1961; 236:1674-9. View

18.

Ratnoff O, Davie E, MALLETT D . Studies on the action of Hageman factor: evidence that activated Hageman factor in turn activates plasma thromboplastin antecedent. J Clin Invest. 1961; 40:803-19. PMC: 290792. DOI: 10.1172/JCI104314. View

19.

DONALDSON V, Rosen F . Hereditary angioneurotic edema: a clinical survey. Pediatrics. 1966; 37(6):1017-27. View

20.

DONALDSON V, Ratnoff O . Hageman factor: alterations in physical properties during activation. Science. 1965; 150(3697):754-6. DOI: 10.1126/science.150.3697.754. View