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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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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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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