Cleaved-C1r HC (R463) Rabbit Polyclonal Antibody

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., 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.