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 ?
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.
Objective: Hereditary angioedema (HAE) is a rare autosomal-dominant disease characterized by recurring attacks of nonpruritic, nonpitting edema caused by an inherited deficiency or dysfunction in the C1 esterase inhibitor (C1 INH).
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.
00) and a slightly elevated C1 esterase inhibitor (C1-INH) level of 0.
Patients have low levels or activity of C1 esterase inhibitor (C1INH), C4, and C1q.
HAE is caused by a deficiency of the C1 esterase inhibitor protein, which is present in blood and helps control inflammation (swelling) and parts of the immune system.
1,2) Hereditary and acquired cases usually result from a deficiency of C1 esterase inhibitor deficiency, which causes an accumulation of bradykinin, leading to soft tissue oedema.
Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond.
This C1 esterase inhibitor complements Shire's FIRAZYR icatibant injection for the treatment of acute HAE attacks.
C1 esterase inhibitor deficiency is a hereditary condition where reduced activity of C1 inhibitor leads to episodic angioedema, often triggered by stress, trauma and surgery.
Halozyme has granted ViroPharma an exclusive worldwide license to use Halozyme's proprietary Enhanze technology, a proprietary drug delivery platform using Halozyme's recombinant human hyaluronidase enzyme (rHuPH20) technology in combination with a C1 esterase inhibitor.