C1S

Complement C1s subcomponent ;
C1 esterase ;
Complement component 1 subcomponent s ;
[Cleaved into: Complement C1s subcomponent heavy chain ;
Complement C1s subcomponent light chain]

catalytic activity:Cleavage of Arg-|-Ala bond in complement component C4 to form C4a and C4b, and Lys(or Arg)-|-Lys bond in complement component C2 to form C2a and C2b: the 'classical' pathway C3 convertase.,disease:Defects in C1S are the cause of selective C1s deficiency [MIM:120580]; that is associated with early onset multiple autoimmune diseases.,enzyme regulation:Inhibited by SERPING1.,function:C1s B chain is a serine protease that combines with C1q and C1s to form C1, the first component of the classical pathway of the complement system. C1r activates C1s so that it can, in turn, activate C2 and C4.,online information:C1S mutation db,PTM:The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.,similarity:Belongs to the peptidase S1 family.,similarity:Contains 1 EGF-like domain.,similarity:Contains 1 peptidase S1 domain.,similarity:Contains 2 CUB domains.,similarity:Contains 2 Sushi (CCP/SCR) domains.,subunit:C1 is a calcium-dependent trimolecular complex of C1q, C1r and C1s in the molar ration of 1:2:2. Activated C1s is an disulfide-linked heterodimer of a heavy chain and a light chain.,

adaptive immune response, immune effector process, activation of immune response, leukocyte mediated immunity,lymphocyte mediated immunity, humoral immune response mediated by circulating immunoglobulin, adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains, acute inflammatory response, activation of plasma proteins involved in acute inflammatory response, positive regulation of immune system process, proteolysis, defense response, inflammatory response, immune response, complement activation, complement activation, classical pathway, humoral immune response, response to wounding, glial cell differentiation, response to organic substance, immunoglobulin mediated immune response, protein processing, B cell mediated immunity, gliogenesis, innate immune response, positive regulation of response to stimulus, positive regulation of immune response, response to cAMP, protein maturation, protein maturation by peptide bond cleavage,