C1-INH, Human, ELISA

C1 inhibitor (C1-INH) is a heavily glycosylated single chain molecule of 500 AA. It inhibits multiple enzymes, including C1s&r of the CP and MASP-1&2 of the LP, plasmin in the fibrinolytic system and Factor XIIa&XIa of the contact and coagulation system. C1-INH is also called C1 esterase inhibitor, due C1s is often cleaved by synthetic esters in spectrophotometry.C1-INH plays an important role in suppression of complement, inflammation and vascular permeability. C1-INH binding of C1 to the catalytic site of both C1r and C1s releases the latter two from the complex. As a result the activation of the complement system is blocked. Binding to MASP blocks function and thereby consumption of C2,3&4. C1-INH spares the AP, leaving part of the innate antibacterial defense intact. Besides, C1-INH can directly bind and neutralize LPS, inhibiting sepsis and endotoxin shock.C1-INH administration is the common treatment for hereditary angioedema (HAE). A disease commonly caused by heterozygous deficiency of C1-INH and leading to low levels of functional C1-INH and recurrent episodes of dermal and submucosal swelling. This is mediated by its ability to control activation of the contact system in inhibiting bradykinin generation and thereby control of vascular permeability.Studies have also shown that acute renal graft rejection causes increased levels of C1rsC1-inhibitor complexes in plasma. The increasing levels of C1rsC1-inhibitor complexes can already be detected in plasma several days before the first clinical signs are present, suggesting acute renal graft rejection. This can therefore be considered as a potential parameter in detecting antibody-mediated kidney rejection.The key advantages:- Short assay time- Developed by complement specialist- Reliable and robust measurement of C1-INH

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