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c1-esterase-inhibitor
C1-Esterase Inhibitor
C1-esterase inhibitor (SERPING1; chr11q12.1) is a serpin blocking complement C1r/C1s and contact activation (FXII, kallikrein, FXIa); deficiency → bradykinin excess → vascular permeability → hereditary angioedema; icatibant (B2R antagonist) and berotralstat are approved.
Entry Metadata
| Field | Value |
|---|---|
| ID | c1-esterase-inhibitor |
| Name | C1-Esterase Inhibitor |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 03-molecular |
Cross-Atlas Connections
Sources
- Cicardi M, Banerji A, Bracho F, et al. Icatibant, a new bradykinin-receptor antagonist, in hereditary angioedema. N Engl J Med. 2010;363(6):532-541. · PubMed 20818873
- Banerji A, Riedl MA, Bernstein JA, et al. Effect of lanadelumab compared with placebo on prevention of hereditary angioedema attacks: a randomized clinical trial. JAMA. 2018;320(20):2108-2121. · PubMed 30480729
- Zuraw BL, Busse PJ, White M, et al. Berotralstat (BCX7353) for the prevention of hereditary angioedema. N Engl J Med. 2021;384(23):2186-2195. · PubMed 34077648