complement C1s
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Introduction
activated by C1r to form C1s(dimer)
Function
- activated by C1r to form C1s(dimer)
- C1s B chain is a serine protease that combines with C1q & C1r to form C1, the first component of the complement classical pathway
- C1r activates C1s so that it can, in turn, activate C2 & C4
- cleavage of Arg-|-Ala bond in complement component C4 to form C4a & C4b, & Lys(or Arg)-|-Lys bond in complement C2 to form C2a & C2b: the 'classical' pathway C3-convertase[5]
- inhibited by SERPING1[5]
- KM=12.3 uM for complement component C2 (at 37 degrees Celsius)[5]
- KM=1.9 uM for complement component C4 (at 37 degrees Celsius)[5]
Structure
- belongs to the peptidase S1 family
- contains 2 CUB domains
- contains 1 EGF-like domain
- contains 1 peptidase S1 domain
- contains 2 Sushi (CCP/SCR) domains
- activated C1s is an disulfide-linked heterodimer of a heavy chain & a light chain[6]
Pathology
More general terms
- complement
- serine protease trypsin family
- complement-activating enzyme
- oligomerizing protein
- inter alpha globulin
Additional terms
Component of
References
- ↑ Baron M, Norman DG, Campbell ID. Protein modules. Trends Biochem Sci. 1991 Jan;16(1):13-7. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/2053133
- ↑ Clinical Diagnosis & Management by Laboratory Methods, J.B. Henry (ed), W.B. Saunders Co., Philadelphia, PA. 1991, pg 832
- ↑ UniProt http://www.uniprot.org/uniprot/P09871.html
- ↑ C1Sbase; Note: C1S mutation db http://bioinf.uta.fi/C1Sbase/
- ↑ 5.0 5.1 5.2 5.3 5.4 Rossi V, Cseh S, Bally I, Thielens NM, Jensenius JC, Arlaud GJ. Substrate specificities of recombinant mannan-binding lectin- associated serine proteases-1 and -2. J Biol Chem. 2001 Nov 2;276(44):40880-7. PMID: https://www.ncbi.nlm.nih.gov/pubmed/11527969
- ↑ 6.0 6.1 Hess D, Schaller J, Rickli EE. Identification of the disulfide bonds of human complement C1s. Biochemistry. 1991 Mar 19;30(11):2827-33. PMID: https://www.ncbi.nlm.nih.gov/pubmed/2007122
- ↑ 7.0 7.1 Dragon-Durey MA, Quartier P, Fremeaux-Bacchi V et al Molecular basis of a selective C1s deficiency associated with early onset multiple autoimmune diseases. J Immunol. 2001 Jun 15;166(12):7612-6. PMID: https://www.ncbi.nlm.nih.gov/pubmed/11390518