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The C1 esterase inhibitor (C1-INH) enzyme plays a role in the classical pathway of the complement cascade, which is a component of the immune system response that acts to protect the human body from a variety of foreign substances. [5]
C1-inhibitor (C1-inh, C1 esterase inhibitor) is a protease inhibitor belonging to the serpin superfamily. [5] Its main function is the inhibition of the complement system (C1r, C1s) to prevent spontaneous activation but also as the major regulator of the contact system (PK, FXIIa, and FXIa).
Acute treatment consists of C1-INH (C1-esterase inhibitor) concentrate from donor blood, which must be administered intravenously. In an emergency, fresh frozen blood plasma, which also contains C1-INH, can also be used. However, in most European countries, C1-INH concentrate is only available to patients who are participating in special ...
However, in hereditary angioedema with C1 inhibitor deficiency, C1 inhibitor is either reduced in quantity and function (type 1) or dysfunctional (type 2), this leads to bradykinin disinhibition and bradykinin mediated activation of bradykinin B1 receptor and bradykinin B2 receptor on endothelial cells (cells lining blood vessels). [7]
In dogs with a liver shunt, some of this blood bypasses the liver, preventing it from filtering toxins and nutrients. This can cause toxins and waste to build up in the body, which can lead to ...
A C1-INH concentrate can be used for angio-oedema (C1-INH deficiency). [2] [3] Pneumococcus and Haemophilus infections can be prevented via immunization. [2] Epsilon-aminocaproic acid could be used to treat hereditary C1-INH deficiency, though the possible side effect of intravascular thrombosis should be weighed. [7]
Lack of regulation of the classical complement pathway through the deficiency in C1-inhibitor results in episodic angioedema. [1] C1-inhibitor defiency can be hereditary or acquired, resulting in hereditary or acquired angioedema. [12] C1-inhibitor plays the role of inactivating C1r and C1s to prevent further downstream classical complement ...
716 317677 Ensembl ENSG00000182326 ENSMUSG00000079343 UniProt P09871 Q8CFG8 RefSeq (mRNA) NM_001734 NM_201442 NM_001346850 NM_173864 RefSeq (protein) NP_001333779 NP_001725 NP_958850 NP_776289 Location (UCSC) Chr 12: 6.99 – 7.07 Mb Chr 6: 124.6 – 124.61 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse Complement component 1s (EC 3.4.21.42, C1 esterase, activated complement C1s ...
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