C1R

Complement C1r subcomponent ;
Complement component 1 subcomponent r ;
[Cleaved into: Complement C1r subcomponent heavy chain ;
Complement C1r subcomponent light chain]

catalytic activity:Selective cleavage of Lys(or Arg)-|-Ile bond in complement subcomponent C1s to form the active form of C1s (EC 3.4.21.42).,function:C1r 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.,polymorphism:Complement component C1r deficiency [MIM:216950] leads to the failure of the classical complement system activation pathway (C1 deficiency). Individuals with C1 deficiency are highly susceptible to infections by microorganisms and have greater risk in developing autoimmune diseases such as systemic lupus erythematosus (SLE).,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. C1r is a dimer of identical chains, each of which is activated by cleavage into two chains, A and B, connected by disulfide bonds.,

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,immunoglobulin mediated immune response, protein processing, B cell mediated immunity, innate immune response,positive regulation of response to stimulus, positive regulation of immune response, protein maturation, protein maturation by peptide bond cleavage,

Secreted .