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The Complement System pp 189–205Cite as

C1 Inhibitor: Quantification and Purification

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Part of the book series: Methods in Molecular Biology ((MIMB,volume 1100))

Abstract

C1 inhibitor is a multipotent serpin capable of inhibiting the classical and the lectin pathways of complement, the fibrinolytic system, and contact/kinin system of coagulation. Deficiency of C1 inhibitor manifest as hereditary angioedema (HAE), an autosomal dominant hereditary disease. Measuring the C1 inhibitor level is of vital importance for the diagnosis of HAE and also for monitoring patients receiving C1 inhibitor for therapy. Determination of the antigenic C1 inhibitor level by the radial immunodiffusion (RID) technique is described in detail in this chapter. The presented purification method of plasma C1 inhibitor is primarily based on its high carbohydrate content and its affinity to the lectin jacalin.

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References

  1. Ratnoff OD, Lepow IH (1957) Some properties of an esterase derived from preparations of the first component of complement. J Exp Med 106:327–343

    Article  PubMed  CAS  Google Scholar 

  2. Zeerleder S (2011) C1-inhibitor: more than a serine protease inhibitor. Semin Thromb Hemost 37:362–374

    Article  PubMed  CAS  Google Scholar 

  3. Davis AE 3rd, Lu F, Mejia P (2010) C1 inhibitor, a multi-functional serine protease inhibitor. Thromb Haemost 104:886–893

    Article  PubMed  CAS  Google Scholar 

  4. Carugati A, Pappalardo E, Zingale LC, Cicardi M (2001) C1-inhibitor deficiency and angioedema. Mol Immunol 38:161–173

    Article  PubMed  CAS  Google Scholar 

  5. Tourangeau LM, Zuraw BL (2011) The new era of C1-esterase inhibitor deficiency therapy. Curr Allergy Asthma Rep 11:345–351

    Article  PubMed  CAS  Google Scholar 

  6. Agostoni A, Aygören-Pürsün E, Binkley KE et al (2004) Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol 114(3 Suppl):S51–S131

    Article  PubMed  Google Scholar 

  7. Bene L, Füst G, Fekete B, Kovács A, Horváth L, Prohászka Z, Miklós K, Pálos G, Daha M, Farkas H, Varga L (2003) High normal serum levels of C3 and C1 inhibitor, two acute-phase proteins belonging to the complement system, occur more frequently in patients with Crohn’s disease than ulcerative colitis. Dig Dis Sci 48:1186–1192

    Article  PubMed  CAS  Google Scholar 

  8. Széplaki G, Varga L, Laki J, Dósa E, Rugonfalvi-Kiss S, Madsen HO, Prohászka Z, Kocsis A, Gál P, Szabó A, Acsády G, Karádi I, Selmeci L, Garred P, Füst G, Entz L (2007) Low c1-inhibitor levels predict early restenosis after eversion carotid endarterectomy. Arterioscler Thromb Vasc Biol 27:2756–2762

    Article  PubMed  Google Scholar 

  9. Pilatte Y, Hammer CH, Frank MM, Fries LF (1989) A new simplified procedure for C1 inhibitor purification. A novel use for jacalin-agarose. J Immunol Methods 120:37–43

    Article  PubMed  CAS  Google Scholar 

  10. Mancini G, Carbonara AO, Heremans JF (1965) Immunochemical quantitation of antigens by single radial immunodiffusion. Immunochemistry 2:235–254

    Article  PubMed  CAS  Google Scholar 

  11. Blanchard GC, Gardner R (1980) Two nephelometric methods compared with a radial immunodiffusion method for the measurement of IgG, IgA and IgM. Clin Biochem 13:84–91

    Article  PubMed  CAS  Google Scholar 

  12. Davis DG, Schaefer DM, Hinchcliff KW, Wellman ML, Willet VE, Fletcher JM (2005) Measurement of serum IgG in foals by radial immunodiffusion and automated turbidimetric immunoassay. J Vet Intern Med 19:93–96

    Article  PubMed  Google Scholar 

  13. McCarthy RC, Fetterhoff TJ, Luckey DW (1987) Three immunoassay methods evaluated for quantifying prealbumin (transthyretin) in serum. Clin Chem 33:1430–1433

    PubMed  CAS  Google Scholar 

  14. Bachorik PS, Cloey TA (1989) Rate immunonephelometry and radial immunodiffusion compared for apolipoproteins AI and B assay. Clin Chem 35:2236–2241

    PubMed  CAS  Google Scholar 

  15. Cloppet H, Francina A, Coquelin H, Boucaud-Maître Y, Hutinel P, Creyssel R (1982) Laser nephelometry and radial immunodiffusion compared for immunoglobulin quantification in pathological sera. Clin Chem 28:180–182

    PubMed  CAS  Google Scholar 

  16. Keyser JW, Fifield R, Watkins GL (1981) Standardization of immunochemical determinations of serum albumin. Clin Chem 27:736–738

    PubMed  CAS  Google Scholar 

  17. Buffone GJ, Lewis SA (1977) Effect of analytical factors on immunochemical reference limits for complement component C3 in serum of a reference pediatric population. Clin Chem 23:994–999

    PubMed  CAS  Google Scholar 

  18. Persson K, Kindmark CO, Pensky J, Naff G (1977) Quantitative measurement of properdin in normal human serum by electroimmunoassay and single radial immunodiffusion. Clin Exp Immunol 29:84–88

    PubMed  CAS  Google Scholar 

  19. Cedzyński M, Madaliński K, Gregorek H, Swierzko AS, Nowicka E, Obtułowicz K, Dzierzanowska-Fangrat K, Wojda U, Rabczenko D, Kawakami M (2008) Possible disease-modifying factors: the mannan-binding lectin pathway and infections in hereditary angioedema of children and adults. Arch Immunol Ther Exp 56:69–75

    Article  Google Scholar 

  20. Drouet C, Alibeu C, Ponard D, Arlaud GJ, Colomb MG (1988) A sensitive method to assay blood complement C1-inhibitor activity. Clin Chim Acta 31:121–130

    Article  Google Scholar 

  21. Starcević D, Jelić-Ivanović Z, Kalimanovska V (1991) Plasma C1 inhibitor in malignant diseases: functional activity versus concentration. Ann Clin Biochem 28(Pt 6):595–598

    PubMed  Google Scholar 

  22. Donaldson VH, Falconieri MW (1993) A modification of an affinity procedure for purification of human C1 inhibitor that provides a homogeneous stable preparation. J Immunol Methods 157:101–104

    Article  PubMed  CAS  Google Scholar 

  23. Beinrohr L, Murray-Rust TA, Dyksterhuis L, Závodszky P, Gál P, Pike RN, Wijeyewickrema LC (2012) Serpins and the complement system. Methods Enzymol 499:55–75

    Article  Google Scholar 

  24. Perkins SJ, Smith KF, Amatayakul S, Ashford D, Rademacher TW, Dwek RA, Lachmann PJ, Harrison RA (1990) Two-domain structure of the native and reactive centre cleaved forms of C1 inhibitor of human complement by neutron scattering. J Mol Biol 214:751–763

    Article  PubMed  CAS  Google Scholar 

  25. Dobó J, Major B, Kékesi KA, Szabó I, Megyeri M, Hajela K, Juhász G, Závodszky P, Gál P (2011) Cleavage of kininogen and subsequent bradykinin release by the complement component: mannose-binding lectin-associated serine protease (MASP)-1. PLoS One 6:e20036

    Article  PubMed  Google Scholar 

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Acknowledgements

The authors gratefully acknowledge the advice and technical help of Ms. Márta Kókai and Ms. Zsuzsa Szendrei. J. Dobó was supported by the János Bolyai Research Fellowship and OTKA grants NK77978 and NK100834.

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Authors and Affiliations

  1. 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary

    Lilian Varga

  2. Institute of Enzymology, RCNS, Hungarian Academy of Sciences, Budapest, Hungary

    József Dobó

Authors
  1. Lilian Varga
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  2. József Dobó
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Editor information

Editors and Affiliations

  1. Division of Infectious Diseases, Brigham and Women’s Hospital, Harvard University Harvard Medical School, Boston, Massachusetts, USA

    Mihaela Gadjeva

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© 2014 Springer Science+Business Media, New York

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Varga, L., Dobó, J. (2014). C1 Inhibitor: Quantification and Purification. In: Gadjeva, M. (eds) The Complement System. Methods in Molecular Biology, vol 1100. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-724-2_16

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  • DOI: https://doi.org/10.1007/978-1-62703-724-2_16

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  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-62703-723-5

  • Online ISBN: 978-1-62703-724-2

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