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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).
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 plays the role of inactivating C1r and C1s to prevent further downstream classical complement activity. [13] [12] C1-inhibitor controls the processes involved in maintaining vascular permeability. As a result, C1-inhibitor levels of less than 50% of the standard lead to increased vascular permeability, characteristic of angioedema ...
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]
Activation of the C1 complex initiates the classical complement pathway. This occurs when C1q binds to antigen-antibody complexes. The antibodies IgM or certain subclasses of IgG complexed with antigens are able to initiate the complement system: a single pentameric IgM can initiate the pathway, while several monomeric IgG molecules are needed. [3]
The classical pathway is inhibited by C1-inhibitor, which binds to C1 to prevent its activation. [35] Another example, is a plasma protein called, Factor H (FH), which has a key role in down-regulating the alternative pathway. [36] Factor H, along with another protein called Factor I, inactivates C3b, the active form of C3. This process ...
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 ...
The C1q domain is a conserved protein domain. C1q is a subunit of the C1 enzyme complex that activates the serum complement system.C1q comprises 6 A, 6 B and 6 C chains.These share the same topology, each possessing a small, globular N-terminal domain, a collagen-like Gly/Pro-rich central region, and a conserved C-terminal region, the C1q domain. [2]