C1 esterase inhibitor

C1 es·ter·ase in·hib·i·tor

an α2-neuraminoglycoprotein that inhibits the enzymatic activity of C1 esterase, the activated first component of complement. A deficiency of this inhibitor results in a lack of inhibition of C1r and C1s leading to uncontrolled activation of the complement cascade and angioedema.

SERPING1

A gene on chromosome 11q12-q13.1 that encodes a highly glycosylated plasma protein which regulates the complement cascade by inhibiting activated C1r and C1s, thereby preventing complement activation.

Molecular pathology
C1-INH/SERPING1 deficiency is associated with hereditary angioneurotic oedema (HANE).
References in periodicals archive ?
Based on drug class, the market has been classified into recombinant coagulation factors and human C1 esterase inhibitor. The recombinant coagulation factors sub-segment has been further sub-segmented into recombinant coagulation factor VIII, recombinant coagulation factor IX, recombinant coagulation factor VIIa, and others.
Hereditary and acquired angioedema: problems and progress: proceedings of the Third C1 Esterase Inhibitor Deficiency Workshop and beyond.
It was reported yesterday that the US Food and Drug Administration (USFDA) has approved the technology transfer of Ireland-based Shire's Cinryze - a C1 esterase inhibitor - drug product manufacturing process to its Vienna, Austria manufacturing site.
Binkley et al., "Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond," The Journal of Allergy and Clinical Immunology, vol.
Li et al., "Escalating doses of C1 esterase inhibitor (CINRYZE) for prophylaxis in patients with hereditary angioedema," Journal of Allergy and Clinical Immunology: In Practice, vol.
Angioedema can be classified into three forms: a) an idiopathic form with unknown pathogenetic mechanisms, b) an extrinsic factor-induced form (due to histamine [IgE- and non IgE- triggered] or kinin-mediated pathophysiology), and c) a hereditary/acquired form associated with C1 esterase inhibitor deficiency or dysfunction (5).
Food and Drug Administration today approved Haegarda, the first C1 Esterase Inhibitor (Human) for subcutaneous (under the skin) administration to prevent Hereditary Angioedema (HAE) attacks in adolescent and adult patients.
with Jordan's group (Cedars-Sinai Medical Center, Los Angeles, CA, USA) reviewed the agents, IVIg, anti-CD20 antibodies (rituximab, obinutuzumab), proteasome inhibitors (bortezomib, carfilzomib), anti-IL-6R blocker (tocilizumab), IgG endopeptidase (Ides[R] produced by Streptococcus pyogenes), blockers of B cell stimulator protein to B cell receptor (belimumab) blockers of C5 to inhibit the complement sequence (eculizumab), C1 esterase inhibitor, and belatacept (CTLA-Ig that can inhibit plasma cells and DSA generation) used in desensitization.
We present a rare case of severe lingual angioedema attributed to acquired C1 esterase inhibitor deficiency in the setting of systemic lupus erythematosus (SLE) which persisted despite utilization of previously described treatment strategies.
Angiotensin-converting enzyme inhibitors-induced angioedema treated by C1 esterase inhibitor concentrate (Berinert[R]): About one case and review of the therapeutic arsenal.
Patients have low levels or activity of C1 esterase inhibitor (C1INH), C4, and C1q.