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complement C1 inhibitor; C1 esterase inhibitor (SERPING1, Cinryze, Berinert)

Function: - C1 inhibitor inhibits: a) complement C1r b) complement C1s c) coagulation factor XIa d) coagulation factor XIIa e) plasmin f) kallikrein Structure: 35% carbohydrate Pathology: - deficiency associated with hereditary angioneurotic edema Pharmacology: - C1 inhibitor (human from plasma); Tradenames: Cinryze, Berinert Indications: - prevention of hereditary angioedema attacks Laboratory: - see ARUP consult [2] Manufacturers: Lev Pharm (Cinryze), CSL Behring (Berinert) Notes: - Cinryze twice weekly IV infusion costs > US $300,000 annually - a subcutaneous C1 inhibitor twice weekly is effective [4]

Interactions

molecular events

Related

C1-esterase inhibitor assay

Specific

C1 Esterase Inhibitor Parenteral recombinant C1 esterase inhibitor; recombinant C1 INH

General

glycoprotein inter alpha globulin serine protease inhibitor; serpin

Properties

COMPARTMENT: plasma MOTIF: glycosylation site serpin motif SITE: C-TERMINAL FOR-BINDING-OF: serine protease INHIBITS: complement C1r complement C1s plasmin kallikrein coagulation factor XIa coagulation factor XIIa SECRETED-BY: hepatocyte

Database Correlations

OMIM 106100 UniProt P05155

References

  1. OMIM :accession 106100
  2. ARUP Consult: Hereditary Angioedema - C1-INH Deficiency The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/hereditary-angioedema
  3. ARUP Consult: C1-INH Deficiency Testing Algorithm The Physician's Guide to Laboratory Test Selection & Interpretation https://arupconsult.com/algorithm/c1-inh-deficiency-testing-algorithm
  4. Longhurst H, Cicardi M, Craig T et al. Prevention of hereditary angioedema attacks with a subcutaneous C1 inhibitor. N Engl J Med 2017 Mar 23; 376:1131 PMID: 28328347 http://www.jwatch.org/na43697/2017/03/23/new-preventive-treatment-hereditary-angioedema