Abstract
When purified human C′1 esterase is injected intradermally in man, increased vascular permeability results. This effect is not blocked by soybean trypsin inhibitor and is not abolished by pretreatment with the antihistamine, pyribenzamine, or by compound 48/80. Thus, the effect is not due to the release of endogenous histamine. The decreased permeability response of individuals with a specific hereditary deficiency of C′2 is evidence for the complement-dependent nature of this reaction. The apparently normal response to intradermal C′1 esterase developed by individuals with an acquired specific deficiency of C′3 suggests that the vasoactive substance may be derived from one of the early reacting complement components. Characteristic attacks of angioedema have been provoked by the intradermal injection of human C′1 esterase in two individuals with hereditary angioneurotic edema. Patients with hereditary angioneurotic edema are unresponsive to intradermal injections of C′1 esterase immediately after attacks.
Full text
PDFImages in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- AUSTEN K. F., SHEFFER A. L. DETECTION OF HEREDITARY ANGIONEUROTIC EDEMA BY DEMONSTRATION OF A REDUCTION IN THE SECOND COMPONENT OF HUMAN COMPLEMENT. N Engl J Med. 1965 Apr 1;272:649–656. doi: 10.1056/NEJM196504012721301. [DOI] [PubMed] [Google Scholar]
- Alper C. A., Levin A. S., Rosen F. S. Beta-1C-globulin: metabolism in glomerulonephritis. Science. 1966 Jul 8;153(3732):180–182. doi: 10.1126/science.153.3732.180. [DOI] [PubMed] [Google Scholar]
- Alper C. A., Rosen F. S. Alper CA, Rosen FS: Studies of the in vivo behavior of human C'3 in normal subjects and patients. J Clin Invest. 1967 Dec;46(12):2021–2034. doi: 10.1172/JCI105691. [DOI] [PMC free article] [PubMed] [Google Scholar]
- BECKER E. L. Concerning the mechanism of complement action. V. The early steps in immune hemolysis. J Immunol. 1960 Mar;84:299–308. [PubMed] [Google Scholar]
- BECKER E. L., KAGEN L. THE PERMEABILITY GLOBULINS OF HUMAN SERUM AND THE BIOCHEMICAL MECHANISM OF HEREDITARY ANGIONEUROTIC EDEMA. Ann N Y Acad Sci. 1964 Aug 27;116:866–873. doi: 10.1111/j.1749-6632.1964.tb52552.x. [DOI] [PubMed] [Google Scholar]
- 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]
- DONALDSON V. H., ROSEN F. S. ACTION OF COMPLEMENT IN HEREDITARY ANGIONEUROTIC EDEMA: THE ROLE OF C'1-ESTERASE. J Clin Invest. 1964 Nov;43:2204–2213. doi: 10.1172/JCI105094. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Dias Da Silva W., Lepow I. H. Complement as a mediator of inflammation. II. Biological properties of anaphylatoxin prepared with purified components of human complement. J Exp Med. 1967 May 1;125(5):921–946. doi: 10.1084/jem.125.5.921. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Donaldson V. H., Rosen F. S. Hereditary angioneurotic edema: a clinical survey. Pediatrics. 1966 Jun;37(6):1017–1027. [PubMed] [Google Scholar]
- GOTOFF S. P., FELLERS F. X., VAWTER G. F., JANEWAY C. A., ROSEN F. S. THE BETA-1C GLOBULIN IN CHILDHOOD NEPHROTIC SYNDROME: LABORATORY DIAGNOSIS OF PROGRESSIVE GLOMERULONEPHRITIS. N Engl J Med. 1965 Sep 2;273:524–529. doi: 10.1056/NEJM196509022731004. [DOI] [PubMed] [Google Scholar]
- HAINES A. L., LEPOW I. H. STUDIES ON HUMAN C'1-ESTERASE. I. PURIFICATION AND ENZYMATIC PROPERTIES. J Immunol. 1964 Mar;92:456–467. [PubMed] [Google Scholar]
- Jensen J. Anaphylatoxin in its relation to the complement system. Science. 1967 Mar 3;155(3766):1122–1123. doi: 10.1126/science.155.3766.1122. [DOI] [PubMed] [Google Scholar]
- KAGEN L. J., LEDDY J. P., BECKER E. L. THE PRESENCE OF TWO PERMEABILITY GLOBULINS IN HUMAN SERUM. J Clin Invest. 1963 Aug;42:1353–1361. doi: 10.1172/JCI104819. [DOI] [PMC free article] [PubMed] [Google Scholar]
- KLEMPERER M. R., GOTOFF S. P., ALPER C. A., LEVIN A. S., ROSEN F. S. ESTIMATION OF THE SERUM BETA-1C GLOBULIN CONCENTRATION: ITS RELATION TO THE SERUM HEMOLYTIC COMPLEMENT TITER. Pediatrics. 1965 May;35:765–769. [PubMed] [Google Scholar]
- KOCH W. Relationship between air temperature and mean radiant temperature in thermal comfort. Nature. 1962 Nov 10;196:587–587. doi: 10.1038/196587a0. [DOI] [PubMed] [Google Scholar]
- Klemperer M. R., Austen K. F., Rosen F. S. Hereditary deficiency of the second component of complement (C'2) in man: further observations on a second kindred. J Immunol. 1967 Jan;98(1):72–78. [PubMed] [Google Scholar]
- Klemperer M. R., Woodworth H. C., Rosen F. S., Austen K. F. Hereditary deficiency of the second component of complement (C'2) in man. J Clin Invest. 1966 Jun;45(6):880–890. doi: 10.1172/JCI105403. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 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]
- LEPOW I. H., RATNOFF O. D., ROSEN F. S., PILLEMER L. Observations on a pro-esterase associated with partially purified first component of human complement (C'1). Proc Soc Exp Biol Med. 1956 May;92(1):32–37. doi: 10.3181/00379727-92-22376. [DOI] [PubMed] [Google Scholar]
- 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]
- LEWIS G. P. PLASMA KININS AND OTHER VASOACTIVE COMPOUNDS IN ACUTE INFLAMMATION. Ann N Y Acad Sci. 1964 Aug 27;116:847–854. doi: 10.1111/j.1749-6632.1964.tb52550.x. [DOI] [PubMed] [Google Scholar]
- Laurell C. B., Niléhn J. E. A new type of inherited serum albumin anomaly. J Clin Invest. 1966 Dec;45(12):1935–1945. doi: 10.1172/JCI105498. [DOI] [PMC free article] [PubMed] [Google Scholar]
- MACKAY M. E., MILES A. A., SCHACHTER M., WILHELM D. L. Susceptibility of the guinea pig to pharmacological factors from its own serum. Nature. 1953 Oct 17;172(4381):714–716. doi: 10.1038/172714b0. [DOI] [PubMed] [Google Scholar]
- MILES A. A., WILHELM D. L. Enzyme-like globulins from serum reproducing the vascular phenomena of inflammation. I. An activable permeability factor and its inhibitor in guinea-pig serum. Br J Exp Pathol. 1955 Feb;36(1):71–81. [PMC free article] [PubMed] [Google Scholar]
- MOTA I. The mechanism of action of anaphylatoxin. Its effect on guinea pig mast cells. Immunology. 1959 Oct;2:403–413. [PMC free article] [PubMed] [Google Scholar]
- NAFF G. B., PENSKY J., LEPOW I. H. THE MACROMOLECULAR NATURE OF THE FIRST COMPONENT OF HUMAN COMPLEMENT. J Exp Med. 1964 Apr 1;119:593–613. doi: 10.1084/jem.119.4.593. [DOI] [PMC free article] [PubMed] [Google Scholar]
- PATON W. D. M. Compound 48/80: a potent histamine liberator. Br J Pharmacol Chemother. 1951 Sep;6(3):499–508. doi: 10.1111/j.1476-5381.1951.tb00661.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
- PENSKY J., LEVY L. R., LEPOW I. H. Partial purification of a serum inhibitor of C'1-esterase. J Biol Chem. 1961 Jun;236:1674–1679. [PubMed] [Google Scholar]
- RATNOFF O. D., LEPOW I. H. COMPLEMENT AS A MEDIATOR OF INFLAMMATION. ENHANCEMENT OF VASCULAR PERMEABILITY BY PURIFIED HUMAN C'1 ESTERASE. J Exp Med. 1963 Nov 1;118:681–698. doi: 10.1084/jem.118.5.681. [DOI] [PMC free article] [PubMed] [Google Scholar]
- RATNOFF O. D., LEPOW I. H. Some properties of an esterase derived from preparations of the first component of complement. J Exp Med. 1957 Aug 1;106(2):327–343. doi: 10.1084/jem.106.2.327. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 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]
- WEBSTER M. E., PIERCE J. V. Action of the kallikreins on synthetic ester substrates. Proc Soc Exp Biol Med. 1961 May;107:186–191. doi: 10.3181/00379727-107-26575. [DOI] [PubMed] [Google Scholar]
- WEBSTER M. E., PIERCE J. V. Studies on plasma kallikrein and its relationship to plasmin. J Pharmacol Exp Ther. 1960 Dec;130:484–491. [PubMed] [Google Scholar]
- WEBSTER M. E., PIERCE J. V. The nature of the kallidins released from human plasma by kallikreins and other enzymes. Ann N Y Acad Sci. 1963 Feb 4;104:91–107. doi: 10.1111/j.1749-6632.1963.tb17655.x. [DOI] [PubMed] [Google Scholar]
- WILHELM D. L., MILES A. A., MACKAY M. E. Enzyme-like globulins from serum reproducing the vascular phenomena of inflammation. II. Isolation and properties of the permeability factor and its inhibitor. Br J Exp Pathol. 1955 Feb;36(1):82–104. [PMC free article] [PubMed] [Google Scholar]