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Human C-1 Esterase Inhibitor
Native whole molecule
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Proteins Synonyms: C1EI, Complement C1 esterase inhibitor, Esterase inhibitor C-1, C1INH, C1-Inhibitor, SERPING1 Source: Human Plasma Purity: ≥ 95% by SDS-PAGE Physical State: Frozen in 20 mM potassium phosphate, pH 7.0, with 250 mM potassium chloride Manufacturer: |
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Athens Research & Technology
Produktdetails
Synonyms
C1EI, Complement C1 esterase inhibitor, Esterase inhibitor C-1, C1INH, C1-Inhibitor, SERPING1
Description of Human C-1 Esterase Inhibitor
Human C-1 Esterase Inhibitor is a 100 kDa protein. C1 esterase inhibitor (C1-INH), belongs to the serine protease inhibitor (SERPIN) superfamily. It regulates proteolytic cascades by irreversibly inhibiting complement components C1r/C1s, coagulation factors XIa/XIIa, plasma kallikrein, and plasmin. Structurally, its N-terminal domain facilitates glycosylation and substrate recognition, while the C-terminal serpin domain mediates protease inhibition via reactive center loop insertion. Normal plasma concentrations range from 16–33 mg/dL, maintaining homeostasis across complement, contact, and fibrinolytic systems. Deficiency or dysfunction of C1-INH causes hereditary angioedema (HAE), with type I (85% of cases) characterized by low protein levels and type II by dysfunctional variants. Mutations in SERPING1 disrupt bradykinin regulation, leading to recurrent edema in subcutaneous tissues (facial swelling), gastrointestinal mucosa (abdominal pain), and upper airways (life-threatening laryngeal edema). Acquired forms arise from autoantibodies against C1-INH or hyperactivation in lymphoproliferative disorders, distinguished by low C1q levels. Therapeutically, plasma-derived (Berinert®, Cinryze®) and recombinant (Ruconest®) C1-INH concentrates are first-line treatments for acute HAE attacks and prophylaxis. Diagnostic evaluation combines functional C1-INH assays, C4/C2 quantification, and genetic testing for SERPING1 variants. These advancements underscore C1-INH’s dual role as a critical immune regulator and therapeutic target in angioedema pathologies.
Source
Human plasma non-reactive for HBsAG, anti-HCV, anti-HBc, and negative for anti-HIV 1 & 2 by FDA approved tests
Storage
For long term, store Human C-1 Esterase Inhibitor at ≤ -80°C.
Applications
Proteolytic Inhibition, Fibrinolysis, Coagulation, In Vitro Diagnostic, Complement System, Immunity, Hemostasis, Biotherapeutics.
Citations/Publications
Tivawala, R., et al., (2018), 'The Rheumatoid Arthritis-Associated Citrullinome', Cell Chemical Biology 25: pp 691–704. Available at: https://doi.org/10.1016/j.chembiol.2018.03.002
Zamolodchikov, D., et al., (2016), 'The Alzheimer’s disease peptide b-amyloid promotes thrombin generation through activation of coagulation factor XII', J Thromb Haemost, 14: pp 995–1007. Available at: DOI: 10.1111/jth.13209.
Sivananthan, S., et al., (2024), 'Prolonging the circulatory half-life of C1 esterase inhibitor via albumin fusion', PLoS ONE 19(10): pp e0305719. Available at: https://doi.org/10.1371/journal.pone.0305719
Tarandovskiy, I. D., et al., (2022), 'C1-inhibitor influence on platelet activation by thrombin receptors agonists', Clinical and Applied Thrombosis/Hemostasis. 2022;28. Available at: doi:10.1177/10760296221120422.
NCBI: https://www.ncbi.nlm.nih.gov/protein/P05155/
Shipped with dry ice
Gel Scan of Human C-1 Esterase Inhibitor
| Fig. 1: SDS-PAGE Gel |
Usage: For research use only. Not for use in diagnostic or therapeutic procedures. Not for human use.
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