Cl inhibitor

Cl inhibitor deficiency causes angioedema as a result of excessive bradykinin production. Thus the pathways and control mechanisms for bradykinin formation and... 

Factor Xlla converts prekallikrein to kallikrein and kallikrein cleaves HK to generate bradykinin. There is also an important positive feedback in the system in which the kallikrein generated rapidly converts unactivated factor XII to activated factor XII, and the rate of this reaction is hundreds of times faster than the rate of autoactivation [11]. Therefore, much of the unactivated factor XII can be cleaved and activated by kallikrein. Cl inhibitor inhibits all functions of factor Xlla and it is one of two major plasma kallikrein inhibitors. Thus all functions of kallikrein are also inhibited, including the feedback activation of factor XII, the cleavage of HK, and the activation of plasma pro-urokinase [66] to lead to plasmin formation. Cl inhibitor also inhibits the fibrinolytic enzyme plasmin, although it is a relatively minor inhibitor compared to a2-antiplasmin or a2-macroglobulin. 

An acquired form of this disease has been described in patients with lymphoma who have circulating low molecular weight IgM and depressed Cl inhibitor levels. This... 

Synthesis of Cl inhibitor in fibroblasts from patients with type I and type II hereditary angioneurotic edema. J Clin Invest 1991 87 1614-1620. 

Caldwell J, Ruddy S, Schur P, Austen K Acquired Cl inhibitor deficiency in lymphosarcoma. Clin Immunol Immunopathol 1972 1 39-52. 

Hauptmann G, Lang J, North M, Oberling F. Mayer G, Lachmann P Acquired Cl-inhibitor deficiencies in lymphoproliferafive diseases with serum immu-noglobuhn abnormalities. A study of three cases. Blut 1976 32 195-206. 

Schreiber A, Zweiman B, Atkins P. Goldwein F, Pietra G, Atkinson B, et al Acquired angioedema with lymphoproliferafive disorder association of Cl inhibitor deficiency with cellular abnormality. Blood 1976 48 567-580. 

Alsenz J, Bork K, Loos M Autoantibody-mediated acquired deficiency of Cl inhibitor. N Engl J Med 1987 316 1360-1366. 

Zuraw B, Curd J Demonstration of modified inactive first component of complement (Cl) inhibitor in the plasmas of Cl inhibitor-deficient patients. J Chn Invest 1986 78 567-575. 

Quastel M, Harrison R, Cicardi M, Alper C, Rosen F Behavior in vivo of normal and dysfunctional Cl inhibitor in normal subjects and patients with hereditary angioneurotic edema. J Chn Invest 1983 71 1041-1046. 

Kramer J, Rosen F, Colten H, Rajczy K, Strunk R Transinhibifion of Cl inhibitor synthesis in type I hereditary angioneurotic edema. J Chn Immunol 1993 91 1258-1262. 

Zuraw B, Sugimoto S, Curd J The value of rocket Immunoelectrophoresis for C4 activation in the evaluation of patients with angioedema or Cl-inhibitor deficiency. J Allergy Clin Immunol 1986 78 1115-1120. 

Activation of factor XII and cleavage of high molecular weight kininogen during acute attacks in hereditary and acquired Cl-inhibitor deficiencies. Immunopharmacology 1996 33 361-364. 

Zahedi R, Bissler J, Davis AR. Andreadis C. Wisnieski J Unique Cl inhibitor dysfunction in a kindred without angioedema. II. Identification of an Ala443 Val substitution and functional analysis 92 of the recombinant mutant protein. J Clin Invest 1995 95 1299-1305. 

Han E, MacFarlane R, Mulhgan A, Scafidi J, Davis AR Increased vascular permeabihty in Cl inhibitor-deficient mice mediated by the bradykinin type 2 receptor. J Clin Invest 2002 8 1057-1063. 

Wallace EM, Perkins SJ, Sim RB, Willis AC, Feighery C, Jackson J Degradation of Cl inhibitor by plasmin implications for the control of inflammatory processes. Mol Med 1997 3 385-396. 

Geha R, Quinti I, Austen K, Cicardi M, Sheffer A, Rosen F Acquired Cl-inhibitor deficiency associated with anti-idiotypic antibody to monoclonal immunoglobuhns. N Engl J Med 1985 312 534-540. Donaldson V, Hess E, McAdams A Lupus-erythe-matosus-like disease in three umelated women with hereditary angioneurotic edema. Arm Intern Med 1977 86 312-313. 

Jackson J, Sim R, Whelan A, Feighery C An IgG autoantibody which inactivates Cl-inhibitor. Nature 1986 323 722-724. 

Jackson J. Sim R. Whaley K, Feighery C Autoantibody facilitated cleavage of Cl-inhibitor in autoimmune angioedema. J Chn Invest 1989 83 698-707. He S. Sim R. Whaley K Mechanism of action of anti-Cl-inhibitor autoantibodies prevention of the formation of stable Cls-Cl-inh complexes. Mol Med 1998 4 119-128. 

Dewald G, Bork K Missense mutations in the coagulation factor XII (Hageman factor) gene in hereditary angioedema with normal Cl inhibitor. Biochem Biophys Res Commun 2006 343 1286-1289. 

Martin L, Raison-Peyron N. Nothen M. Cichon S. Drouet C Hereditary angioedema with normal Cl inhibitor gene in a family with affected women and men is associated with the p.Thr328Lys mutation in theP12gene.J Allergy Chn Immunol 2007 120 975-977. 

Cl, C2...C9 The 9 main components of complement Cl inhibitor A serine protease inhibitor which inactivates Clr/Cls Clq Complement fragment Iq ClqR Receptor for Clw facilitates attachment of immune complexes to mononuclear leucocytes and endothelium... 

Inhibitors that block the protease activity of F-XIIa and kallikrein. Plasma contains two protease inhibitors that regulate contact activation Cl inhibitor (Cl-Inh) and a2-macroglobulin (a2-M). 

N9. Nuijens, J. H., Huijbregts, C. C., Eerenberg-Belmer, A. J., Abbink, J. J Strack Van Schijndel, R. J., Felt-Bersma, R. J., Thijs, L. G., and Hack, C. E Quantification of plasma factor Xlla-Cl-inhibitor and kallikrein-C-inhibitor complexes in sepsis. Blood 72, 1841-1848 (1988). 

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