C1 inhibitor: quantification and purification.
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.
3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
Lilian
L
Varga
József
J
Dobó
D002846:Chromatography, Affinity; D003174:Complement C1 Inactivator Proteins / Q000276:immunology / Q000302:isolation & purification* / Q000378:metabolism*; D050718:Complement C1 Inhibitor Protein; D006801:Humans; D005779:Immunodiffusion / Q000379:methods
D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; C469952:SERPING1 protein, human