C-1 Esterase Inhibitor [80295-38-1]

Athens Research

C-1 Esterase Inhibitor [80295-38-1]

9

Proteolytic Inhibition, Fibrinolysis, Coagulation, In Vitro Diagnostic, Complement System, Immunity, Hemostasis, Biotherapeutics

Research Use Only

≥95% by SDS-PAGE

Plasma protease C1 inhibitor

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.

more then 1 year

Source human plasma non-reactive for HBsAG, anti-HCV, anti-HBc, and negative for anti-HIV 1 & 2 by FDA approved tests.

≤ -80° C

41116100