||Conditioning||Package size ||
|4-5345003||kit||30 to 60 ||
Reagent kit for the chromogenic determination of C1 esterase inhibitor (C1-INH). Defect in the synthesis of C1-INH leads to hereditary angioedema.
The chromogenic activity reagent is used for the diagnosis of angioneurotic edema and for monitoring substitution or steroid therapy in angioneurotic edema. The C1-esterase inhibitor (C1-INH) is a regulatory protein that functions as an inhibitor of several serine proteases in the complement system, the kallikrein-kinin system, the coagulation cascade and in fibrinolysis.
- 1 vial of Substrate C1 (3 mL)
- 1 vial of C1-Esterase (3 mL)
- 1 vial of Sample Buffer A (25 mL)
- 1 vial of Reaction Buffer B (20 mL)
- 1 vial of Coagulation Reference (1 mL)
- 1 vial of Coagulation Control A (1 mL)
- 1 vial of Coagulation Control N (1 mL)
C1-inhibitor (C1-inh, C1 esterase inhibitor) is a protease inhibitor belonging to the serpin superfamily. Its main function is the inhibition of the complement system to prevent spontaneous activation. C1-inhibitor is an acute-phase protein that circulates in blood at levels of around 0.25 g/L. The levels rise ≈ 2-fold during inflammation. C1-inhibitor irreversibly binds to and inactivates C1r and C1s proteases in the C1 complex of classical pathway of complement. MASP-1 and MASP-2 proteases in MBL complexes of the lectin pathway are also inactivated. This way, C1-inhibitor prevents the proteolytic cleavage of later complement components C4 and C2 by C1 and MBL. Although named after its complement inhibitory activity, C1-inhibitor also inhibits proteases of the fibrinolytic, clotting, and kinin pathways. C1-inhibitor is the most important physiological inhibitor of plasma kallikrein, fXIa, and fXIIa.