C1EstheraseInhibitors

C1 Esterase Inhibitors are biological substances that play a critical role in regulating the complement system, part of the body's immune response. These inhibitors primarily target C1 esterase, a protein involved in the activation of the classical pathway of the complement system. By doing so, they help prevent excessive inflammation and tissue damage resulting from uncontrolled complement activation.

The most well-known C1 esterase inhibitor is C1 esterase inhibitor (C1-INH), a glycoprotein encoded by the SERPING1 gene. It is naturally occurring in the plasma and is also available as a therapeutic agent. Deficiency or dysfunction of C1-INH can lead to hereditary angioedema (HAE), a condition characterized by recurrent episodes of severe swelling, often affecting the face, extremities, gastrointestinal tract, and airways.

Clinically, C1-INH concentrates are used to treat and prevent angioedema attacks in patients with hereditary angioedema. These concentrates can be derived from human plasma or produced synthetically through recombinant DNA technology. Beyond hereditary angioedema, C1-INH therapy is also investigated for other inflammatory and autoimmune conditions where complement activation plays a significant role.

In addition to therapeutic applications, research into C1 esterase inhibitors provides insights into immune regulation and potential targets for managing immune-mediated diseases. The regulation of the complement system by C1-INH exemplifies the complex balance necessary for immune defense while preventing self-damage.