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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]
Normal C1 inhibitor level hereditary angioedema is thought to involve various mutations that increased bradykinin activity and cause a decreased threshold for activation of the plasma contact system thus leading to the symptoms of angioedema. [7] Hereditary angioedema with normal C1-inhibitor is a genetically heterogeneous disorder.
Santarus and Pharming Announce Poster Presentation of Pivotal Clinical Data for RUCONEST (Recombinant Human C1 Esterase Inhibitor) SAN DIEGO & LEIDEN, Netherlands--(BUSINESS WIRE)-- Santarus, Inc ...
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 ...
Disorders of the proteins that act to inhibit the complement system (such as C1-inhibitor) can lead to an overactive response, causing conditions such as hereditary angioedema. [8] Disorders of the proteins that act to activate the complement system (such as C3) can lead to an underactive response, causing greater susceptibility to infections. [9]
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 main type for that purpose is acetylcholinesterase (also called choline esterase I [2] or erythrocyte cholinesterase); it is found mainly in chemical synapses and red blood cell membranes. The other type is butyrylcholinesterase (also called choline esterase II [2] or plasma cholinesterase); it is found mainly in the blood plasma.
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