Human C1-esterase inhibitor

Human C1-esterase inhibitor is a protein that functions as a key down-regulator of the complement and contact activation systems. It can be derived from human plasma or produced recombinantly. It is approved for the treatment of acute attacks and prophylaxis of hereditary angioedema (HAE), a condition caused by C1 inhibitor deficiency or dysfunction. The drug works by inhibiting complement activation, which is implicated in the pathophysiology of HAE and other inflammatory conditions. It is also being investigated for its potential to ameliorate adverse drug reactions related to IVIG infusions, treat acute exacerbations of neuromyelitis optica (NMO), and has shown efficacy in preclinical models for conditions like hemorrhagic shock and immune-mediated hemolytic anemia.

Brand names

RUCONESTCINRYZEHAEGARDABerinert

Other names

C1-esterase inhibitor (human)C1-esterase inhibitor concentrateC-1-esterase inhibitor concentrateC 1-esterase inhibitor concentratePurified human C1-esterase inhibitorPlasma-derived human C1-esterase inhibitor