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 ?
Contract notice: Tender for the supply of C1 esterase inhibitor.
RUCONEST (conestat alfa) is a recombinant human C1 esterase inhibitor approved for the treatment of angioedema attacks in patients with HAE in the US, Israel, all 28 EU countries plus Norway, Iceland and Liechtenstein.
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.
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.
Therapeutic use: Human C1 esterase inhibitor (7, 8) is indicated for the treatment of acute abdominal or facial attacks of hereditary angioedema (HAE) in adult and adolescent patients.
Gupta S, Yu F, Klaustermeyer WB: New-variant hereditary angioedema in three brothers with normal C1 esterase inhibitor level and function.
The literature search was performed using Pubmed, and the terms Africa and African and Zulu in combination with each of C1 esterase inhibitor, hereditary angio-oedema and hereditary angioedema.
The complement protein C1 esterase inhibitor (C1-INH) is an acute phase protein [13], the plasma level of which can possibly increase in acute phase responses.
The condition is caused by a genetic deficiency of C1 esterase inhibitor (C1-INH), a naturally occurring molecule that inhibits kallikrein and other serine proteases in the blood.
Hereditary angioedema is caused by an absolute deficiency or the functional inactivity of C1 esterase inhibitor in plasma.
RUCONESTA (conestat alfa) is a recombinant human C1 esterase inhibitor approved for the treatment of angioedema attacks in patients with HAE in the US, Israel, all 28 EU countries plus Norway, Iceland and Liechtenstein.
Do not take RUCONEST if you have a history of life-threatening immediate allergic reactions to C1 esterase inhibitor preparations, including anaphylaxis.