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Target intelligence / Profile preview
Complement C1 Inhibitor (C1-INH)
- Target
- C1-INH
- Molecular classification
- Serine protease inhibitor, Serpin
01
Overview
Complement C1 inhibitor (C1-INH) is a serine protease inhibitor (serpin) that regulates the complement system, contact system, coagulation pathway, and fibrinolytic pathway. It inhibits activation of complement components (C1r, C1s) to prevent spontaneous or excessive activation of the classical pathway. Deficiency or dysfunction of C1-INH causes hereditary angioedema (HAE). C1-INH is used as replacement therapy for prophylaxis/treatment of hereditary angioedema attacks.
- Other names
- C1 esterase inhibitorC1InhSERPING1
02
Mechanism of action
Irreversible binding to and inhibition of target proteases (C1s, kallikrein, factor XIIa/XIa, MASP2, plasmin, tissue plasminogen activator), preventing their activity.
03
Biological functions
Complement pathway regulationContact system regulationCoagulation pathway regulationFibrinolytic pathway regulationRegulation of vascular permeabilityInflammation regulation
04
Disease associations
Hereditary Angioedema (HAE)Age-related macular degenerationAutoimmune diseases (e.g., lupus erythematosus)
05
Safety considerations
Risk of thromboembolic events with some C1-INH concentratesPotential for allergic reactions
06
Interacting drugs
Berinert®
2 more in the full profile.
07
Biomarkers
C1-INH levels (for HAE diagnosis)C1-INH functional activity (for HAE diagnosis)
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