Gosset C1 esterase inhibitor Overview
C1 esterase inhibitor (C1-INH) is a serine protease inhibitor (serpin) that plays a critical role in regulating the complement system, contact system, and coagulation/fibrinolytic pathways. It inhibits the activation of the classical complement pathway by binding to and inactivating proteases such as C1r and C1s. C1-INH also regulates the contact system by inhibiting plasma kallikrein, factor XIIa, and factor XIa. Deficiency or dysfunction of C1-INH leads to unchecked complement activation and increased bradykinin levels, resulting in conditions like hereditary angioedema (HAE).
Mechanism of Action
Irreversibly binds to and inhibits target proteases (C1s, kallikrein, factor XIIa/XIa), preventing their enzymatic activity and downstream effects like bradykinin production and complement activation.
Biological Functions
Disease Associations
Safety Considerations
- Risk of acute edema/swelling affecting subcutaneous tissue, GI tract, or upper airway, potentially life-threatening if untreated.
- Potential complications associated with plasma-derived therapies.
Interacting Drugs
Associated Biomarkers
| Biomarker |
|---|
| Antigenic level of C1-INH |
| Functional activity of C1-INH |