Journal of Clinical Immunology

, Volume 2, Issue 2, pp 65–68 | Cite as

The C1 esterase inhibitor and hereditary angioedema

  • Michael M. Frank
Special Article

Key words

C1 esterase inhibitor angioedema 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Pensky J, Schwick HG: Human serum inhibitor of C'1 esterase: Identity with α2-neuraminoglycoprotein. Science 163:689, 1969Google Scholar
  2. 2.
    Lepow IH, Ratnoff OD, Rosen FS, Pillemer L: Observations on a proesterase associated with partially purified first component of human complement (C'1). Proc Soc Exp Biol Med 92:32, 1956Google Scholar
  3. 3.
    Ratnoff OD, Lepow IH: Some properties of an esterase derived from preparations of the first component of complement. J Exp Med 106:327, 1957Google Scholar
  4. 4.
    Lepow IH, Ratnoff OD, Levy LR: Studies on the activation of a proesterase associated with partially purified first component of human complement. J Exp Med 107:451, 1958Google Scholar
  5. 5.
    Pensky J, Levy LR, Lepow IH: Partial purification of a serum inhibitor of C'1-esterase. J Biol Chem 236:1674, 1961Google Scholar
  6. 6.
    Landerman NS, Webster ME, Becker ED, Ratcliffe HE: Hereditary angioneutotic edema. II. Deficiency of inhibitor serum globulin permeability factor and/or plasma kallikrein. J Allergy 33:330, 1962Google Scholar
  7. 7.
    Donaldson VH, Evans RR: A biochemical abnormality in hereditary angioneurotic edema. Am J Med 35:37, 1963Google Scholar
  8. 8.
    Osler W: Hereditary angio-neurotic edema. Am J Med Sci 95:362, 1888Google Scholar
  9. 9.
    Rosen FS, Charache PJ, Pensky J, Donaldson V: Hereditary angioneurotic edema: Two genetic variants. Science 148:957, 1965Google Scholar
  10. 10.
    Rosen FS, Alper CA, Pensky J, Klemperer MR, Donaldson VH: Genetically determined heterogeneity of C1 esterase inhibitor in patients with hereditary angioneurotic edema. J Clin Invest 50:2143, 1971Google Scholar
  11. 11.
    Vaughan VC, McKay RJ, Nelson WE (eds): Textbook of Pediatrics, 10th ed. Philadelphia, W.B. Saunders, 1975, p 407Google Scholar
  12. 12.
    Harpel PC, Cooper NR: Studies on human plasma C1-inactivator-enzyme interactions. I Mechanisms of interaction with C1s, plasmin and trypsin. J Clin Invest 55:593, 1975Google Scholar
  13. 13.
    Haupt H, Heimburg N, Kranz T, Schnick HG: Ein Bertrag zur Isolierung and Charakterisierung des C1-Inaktivators aus Human Plasma. Eur J Biochem 17:254, 1970Google Scholar
  14. 14.
    Minta JO: The role of sialic acid in the functional activity and the hepatic clearance of C1-INH. J Immunol 126:245, 1981Google Scholar
  15. 15.
    Johnson AM, Alper CH, Rosen FS: C1 inhibitor—evidence for decreased hepatic synthesis in hereditary angioneurotic edema. Science 173:553, 1971Google Scholar
  16. 16.
    Gadek JE, Hosea SW, Gelfand JA, Frank MM: Response of variant hereditary angioedema phenotypes to danazol therapy. J Clin Invest 64:280, 1979Google Scholar
  17. 17.
    Curd JG, Yelvington M, Ziccardi R, Mathison DA, Griffin JH: Purification and characterization of two functionally distinct forms of C1 inhibitor from a patient with angioedema. Clin Exp Immunol 45:261, 1981Google Scholar
  18. 18.
    Laurell AB, Martensson U: C1 inactivator protein complexed with albumin in plasma from a patient with angioneurotic edema. Eur J Immunol 1:146, 1971Google Scholar
  19. 19.
    Forbes CG, Pensky J, Ratnoff OD: Inhibition of activated Hageman factor and activated thromboplastin antecedent by purified C1 inactivator. J Lab Clin Med 76:809, 1970Google Scholar
  20. 20.
    Ratnoff OD, Pensky J, Ogston D, Naff GB: The inhibition of plasmin, plasma kallikrein, plasma permeability factor and the C1r subcomponent of the first component of complement by serum C1 esterase inhibitor. J Exp Med 129:315, 1969Google Scholar
  21. 21.
    Schreiber AD, Kaplan AP, Austen KF: Inhibition by C1 INH of Hageman factor fragment activation of coagulation, fibrinolysis and kinin generation. J Clin Invest 52:1402, 1973Google Scholar
  22. 22.
    Gigli I, Mason JW, Colman RW, Austen KF: Interaction of plasma kallikrein with the C1 inhibitor. J Immunol 104:574, 1970Google Scholar
  23. 23.
    Movat HZ: Inflammation, Immunity and Hypersensitivity, Hagerstown, MD, Harper & Row, 1979, p 59Google Scholar
  24. 24.
    Donaldson VH, Rosen FS: Action of complement in hereditary angioedema: The role of C1-esterase. J Clin Invest 43:2204, 1964Google Scholar
  25. 25.
    Müller-Eberhard HJ: Complement. Ann Rev Biochem 44:697, 1975Google Scholar
  26. 26.
    Austen KF, Sheffer AL: Detection of hereditary angioneurotic edema by demonstration of a reduction in the second component of human complement. N Engl J Med 272:649, 1965Google Scholar
  27. 27.
    Donaldson VH, Rosen FS, Bing DH: Role of the second component of complement (C2) and plasmin in kinin release in hereditary angioneurotic edema (H.A.N.E.) plasma. Trans Am Assoc Phys XL:174, 1977Google Scholar
  28. 28.
    Curd JG, Prograis LJ Jr, Cochrane CA: Detection of active kallikrein in induced blister fluids of hereditary angioedema patients. J Exp Med 152:742, 1980Google Scholar
  29. 29.
    Gigli I, Ruddy S, Austen KF: The stoichiometric measurement of the serum inhibitor of the first component of complement by the inhibition of immune hemolysis. J Immunol 100:1154, 1968Google Scholar
  30. 30.
    Gelfand JA, Sherins RJ, Alling DW, Frank MM: The treatment of hereditary angioedema with danazol. Reversal of clinical and biochemical abnormalities. N Engl J Med 295:1444, 1976Google Scholar
  31. 31.
    Gelfand JA, Boss GR, Conley CL, Reinhart R, Frank MM: Acquired C1 esterase inhibitor deficiency and angioedema: A review. Medicine 58:321, 1979Google Scholar
  32. 32.
    Sim RB, Arlaud GN, Colomb MG: Kinetics of reaction of human C1-inhibitor with the human complement system proteases Clr and Cls. Biochim Biophys Acta 612:433, 1980Google Scholar
  33. 33.
    Ziccardi RJ: Activation of the early components of the classical complement pathways under physiologic conditions. J Immunol 126:1769, 1981Google Scholar
  34. 34.
    Naff GB, Pensky J, Lepow IH: The macromolecular nature of the first component of human complement. J Exp Med 119:593, 1964Google Scholar
  35. 35.
    Ziccardi RJ, Cooper NR: Physicochemical and functional characterization of the C1r subunit of the first complement component. J Immunol 116:496, 1976Google Scholar
  36. 36.
    Sakai K, Stroud RM: Purification, molecular properties and activation of C1 proesterase, C1s. J Immunol 110:1010, 1973Google Scholar
  37. 37.
    Ziccardi RJ, Cooper NR: Active disassembly of the first complement component, C1, by C1-Inactivator. J Immunol 123:788, 1979Google Scholar
  38. 38.
    Sim RB, Arlaud GJ, Colomb MG: C1 inhibitor-dependent dissociation of human complement component C1 bound to immune complexes. Biochem J 179:449, 1979Google Scholar
  39. 39.
    Frank MM, Gelfand JA, Atkinson JP: Hereditary angioedema: The clinical syndrome and its management. Ann Intern Med 84:580, 1976Google Scholar
  40. 40.
    Hosea SW, Santaella ML, Brown EJ, Berger M, Katusha K, Frank MM: Long term therapy of hereditary angioedema with danazol. Ann Intern Med 93:809, 1980Google Scholar
  41. 41.
    Jaffe CJ, Atkinson JP, Gelfand JA, Frank MM: Hereditary angioedema: The use of fresh frozen plasma for prophylaxis in patients undergoing oral surgery. J Allergy Clin Immunol 53:386, 1975Google Scholar
  42. 42.
    Gadek JE, Hosea SW, Gelfand JA, Santaella M, Wickerhauser M, Triantaphyllopoulos DC, Frank MM: Replacement therapy in hereditary angioedema. N Engl J Med 302:542, 1980Google Scholar

Copyright information

© Plenum Publishing Corporation 1982

Authors and Affiliations

  • Michael M. Frank
    • 1
  1. 1.National Institute of Allergy and Infectious Diseases, National Institutes of HealthBethesda

Personalised recommendations