| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
Vol. 52, Issue 1, 91-112, March 2000
Central Haematology Laboratory, University Hospital,
Inselspital, Bern, Switzerland (C.C., S.Z., M.R.); Division of
Haematology, Departement of Internal Medicine, Kantonsspital, Lucerne,
Switzerland (W.A.W.); Central Laboratory of the Netherlands Red
Cross Blood Transfusion Service and Department of Internal
Medicine, Academic Hospital of the Free University Amsterdam,
Amsterdam, The Netherlands (C.E.H.); and Solvay Pharmaceuticals,
Hannover, Germany (B.E.)
I. Introduction
II. Biochemistry and Biology of C1-Inh
A. Biochemistry
B. Synthesis
C. Genetics
D. Interaction with Target Proteinases
E. Inactivation
F. Half-Life and Clearance
III. C1-Inh As Inactivator of Plasma Cascade Systems and Leukocytes
A. Complement System
B. Contact Activation
C. Intrinsic Pathway of Coagulation
D. Fibrinolytic System
E. Leukocytes
IV. Potentiation of C1-Inh Activity by Glycosaminoglycans
V. C1-Inh Therapy in Animal Models and Clinical Disease
A. Sepsis
B. Vascular Leak Syndrome
C. Acute Myocardial Infarction
D. Other Diseases
VI. CRP-Mediated Complement Activation: A Common Target for C1-Inh
Therapy?
VII. Summary
Acknowledgment
References
C1-esterase inhibitor (C1-Inh) therapy was introduced in clinical medicine about 25 years ago as a replacement therapy for patients with hereditary angioedema caused by a deficiency of C1-Inh. There is now accumulating evidence, obtained from studies in animals and observations in patients, that administration of C1-Inh may have a beneficial effect as well in other clinical conditions such as sepsis, cytokine-induced vascular leak syndrome, acute myocardial infarction, or other diseases. Activation of the complement system, the contact activation system, and the coagulation system has been observed in these diseases. A typical feature of the contact and complement system is that on activation they give rise to vasoactive peptides such as bradykinin or the anaphylatoxins, which in part explains the proinflammatory effects of either system. C1-Inh, belonging to the superfamily of serine proteinase inhibitors (serpins), is a major inhibitor of the classical complement pathway, the contact activation system, and the intrinsic pathway of coagulation, respectively. It is, therefore, endowed with anti-inflammatory properties. However, inactivation of C1-Inh occurs locally in inflamed tissues by proteolytic enzymes (e.g., elastase) released from activated neutrophils or bacteria thereby leading to increased local activation of the various host defense systems. Here we will give an overview on the biochemistry and biology of C1-Inh. We will discuss studies addressing therapeutic administration of C1-Inh in experimental and clinical conditions. Finally, we will provide an explanation for the therapeutic benefit of C1-Inh in so many different diseases.
This article has been cited by other articles:
|
|
 |
|
  E. Shagdarsuren, K. Bidzhekov, Y. Djalali-Talab, E. A. Liehn, M. Hristov, R. A. Matthijsen, W. A. Buurman, A. Zernecke, and C. Weber C1-Esterase Inhibitor Protects Against Neointima Formation After Arterial Injury in Atherosclerosis-Prone Mice Circulation, January 1, 2008; 117(1): 70 - 78. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. Szeplaki, L. Varga, J. Laki, E. Dosa, S. Rugonfalvi-Kiss, H. O. Madsen, Z. Prohaszka, A. Kocsis, P. Gal, A. Szabo, et al. Low C1-Inhibitor Levels Predict Early Restenosis After Eversion Carotid Endarterectomy Arterioscler. Thromb. Vasc. Biol., December 1, 2007; 27(12): 2756 - 2762. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. Beinrohr, V. Harmat, J. Dobo, Z. Lorincz, P. Gal, and P. Zavodszky C1 Inhibitor Serpin Domain Structure Reveals the Likely Mechanism of Heparin Potentiation and Conformational Disease J. Biol. Chem., July 20, 2007; 282(29): 21100 - 21109. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. Pillai, J. Sha, T. E. Erova, A. A. Fadl, B. K. Khajanchi, and A. K. Chopra Molecular and Functional Characterization of a ToxR-Regulated Lipoprotein from a Clinical Isolate of Aeromonas hydrophila Infect. Immun., July 1, 2006; 74(7): 3742 - 3755. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N. W. G. Lambrecht, I. Yakubov, C. Zer, and G. Sachs Transcriptomes of purified gastric ECL and parietal cells: identification of a novel pathway regulating acid secretion Physiol Genomics, March 13, 2006; 25(1): 153 - 165. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S J Semple, L L Smith, A J McKune, J Hoyos, B Mokgethwa, A F San Juan, A Lucia, and A A Wadee Serum concentrations of C reactive protein, {alpha}1 antitrypsin, and complement (C3, C4, C1 esterase inhibitor) before and during the Vuelta a Espana Br. J. Sports Med., February 1, 2006; 40(2): 124 - 127. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Zeerleder, C. E. Hack, and W. A. Wuillemin Disseminated Intravascular Coagulation in Sepsis Chest, October 1, 2005; 128(4): 2864 - 2875. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. L. Hart, K. A. Ceonzo, L. A. Shaffer, K. Takahashi, R. P. Rother, W. R. Reenstra, J. A. Buras, and G. L. Stahl Gastrointestinal Ischemia-Reperfusion Injury Is Lectin Complement Pathway Dependent without Involving C1q J. Immunol., May 15, 2005; 174(10): 6373 - 6380. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Cai, V. S. Dole, W. Bergmeier, J. Scafidi, H. Feng, D. D. Wagner, and A. E. Davis III A Direct Role for C1 Inhibitor in Regulation of Leukocyte Adhesion J. Immunol., May 15, 2005; 174(10): 6462 - 6466. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. E. Grys, M. B. Siegel, W. W. Lathem, and R. A. Welch The StcE Protease Contributes to Intimate Adherence of Enterohemorrhagic Escherichia coli O157:H7 to Host Cells Infect. Immun., March 1, 2005; 73(3): 1295 - 1303. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. O-charoenrat, V. Rusch, S. G. Talbot, I. Sarkaria, A. Viale, N. Socci, I. Ngai, P. Rao, and B. Singh Casein Kinase II Alpha Subunit and C1-Inhibitor Are Independent Predictors of Outcome in Patients with Squamous Cell Carcinoma of the Lung Clin. Cancer Res., September 1, 2004; 10(17): 5792 - 5803. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. G. De Simoni, E. Rossi, C. Storini, S. Pizzimenti, C. Echart, and L. Bergamaschini The Powerful Neuroprotective Action of C1-Inhibitor on Brain Ischemia-Reperfusion Injury Does Not Require C1q Am. J. Pathol., May 1, 2004; 164(5): 1857 - 1863. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. W. Lathem, T. Bergsbaken, and R. A. Welch Potentiation of C1 Esterase Inhibitor by StcE, a Metalloprotease Secreted by Escherichia coli O157:H7 J. Exp. Med., April 19, 2004; 199(8): 1077 - 1087. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. Koles, P. H. C. van Berkel, F. R. Pieper, J. H. Nuijens, M. L.M. Mannesse, J. F.G. Vliegenthart, and J. P. Kamerling N- and O-glycans of recombinant human C1 inhibitor expressed in the milk of transgenic rabbits Glycobiology, January 1, 2004; 14(1): 51 - 64. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Zeerleder, C. Caliezi, G. van Mierlo, A. Eerenberg-Belmer, I. Sulzer, C. E. Hack, and W. A. Wuillemin Administration of C1 Inhibitor Reduces Neutrophil Activation in Patients with Sepsis Clin. Vaccine Immunol., July 1, 2003; 10(4): 529 - 535. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. de Zwaan, A.H. Kleine, J.H.C. Diris, J.F.C. Glatz, H.J.J. Wellens, P.F.W. Strengers, M. Tissing, C.E. Hack, M.P. van Dieijen-Visser, and W.T. Hermens Continuous 48-h C1-inhibitor treatment, following reperfusion therapy, in patients with acute myocardial infarction Eur. Heart J., November 1, 2002; 23(21): 1670 - 1677. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T. Sulikowski, B. A. Bauer, and P. A. Patston {alpha}1-Proteinase inhibitor mutants with specificity for plasma kallikrein and C1s but not C1 Protein Sci., September 1, 2002; 11(9): 2230 - 2236. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Roos, A. J. Nauta, D. Broers, M. C. Faber-Krol, L. A. Trouw, J. W. Drijfhout, and M. R. Daha Specific Inhibition of the Classical Complement Pathway by C1q-Binding Peptides J. Immunol., December 15, 2001; 167(12): 7052 - 7059. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Jiang, E. Wagner, H. Zhang, and M. M. Frank Complement 1 Inhibitor Is a Regulator of the Alternative Complement Pathway J. Exp. Med., December 3, 2001; 194(11): 1609 - 1616. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Buerke, H. Schwertz, W. Seitz, J. Meyer, and H. Darius Novel Small Molecule Inhibitor of C1s Exerts Cardioprotective Effects in Ischemia-Reperfusion Injury in Rabbits J. Immunol., November 1, 2001; 167(9): 5375 - 5380. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. J. Walport Complement- Second of Two Parts N. Engl. J. Med., April 12, 2001; 344(15): 1140 - 1144. [Full Text] [PDF]
|
|
|
|
 |
|
 H. Tapper and H. Herwald Modulation of hemostatic mechanisms in bacterial infectious diseases Blood, October 1, 2000; 96(7): 2329 - 2337. [Full Text] [PDF]
|
|
Read all eLetters
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
 |
 |
 |
|
 |
 |
 |
