C-1 Esterase Inhibitor
Product Sizes
1 mg
16-16-031509-1MG
About this Product
- SKU:
- 16-16-031509
- Additional Names:
- C!EI, Complement C1 esterase inhibitor, Esterase inhibitor C-1, C1INH, C1-Inhibitor, SERPING1|Proteolytic Inhibition, Fibrinolysis, Coagulation, In Vitro Diagnostic, Complement System, Immunity, Hemostasis, Biotherapeutics
- Buffer:
- Frozen in 20 mM potassium phosphate, pH 7.0, with 250 mM potassium chloride
- CAS Number:
- 80295-38-1
- CE/IVD:
- RUO
- Extra Details:
- 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.
- Formulation:
- Frozen in 20 mM potassium phosphate, pH 7.0, with 250 mM potassium chloride
- Molecular Weight:
- 100,000 Da
- Physical State:
- Frozen
- Purity:
- ≥95%
- Purification:
- Liquid Chromatography Methods
- Shipping Conditions:
- Dry Ice
- Source:
- Source human plasma non-reactive for HBsAG, anti-HCV, anti-HBc, and negative for anti-HIV 1 & 2 by FDA approved tests.
- Storage Conditions:
- Please refer to datasheet
- Supplier:
- Athens Research & Technology Inc.
- Type:
- Proteins, Peptides, Small Molecules & Other Biomolecules: Native
