MGP Database

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MGP000351

UniProt Annotations

Entry Information
Gene Namecomplement component 1, r subcomponent
Protein EntryC1R_HUMAN
UniProt IDP00736
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivitySelective cleavage of Lys(or Arg)-|-Ile bond in complement subcomponent C1s to form the active form of C1s (EC 3.4.21.42).
FunctionC1r 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.
InteractionP09871:C1S; NbExp=3; IntAct=EBI-3926504, EBI-2810045;
PolymorphismComplement 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).
PtmThe iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains. {ECO:0000269|PubMed:2820791}.
SimilarityBelongs to the peptidase S1 family. {ECO:0000255|PROSITE-ProRule:PRU00274}.
SimilarityContains 1 EGF-like domain. {ECO:0000305}.
SimilarityContains 1 peptidase S1 domain. {ECO:0000255|PROSITE- ProRule:PRU00274}.
SimilarityContains 2 CUB domains. {ECO:0000255|PROSITE- ProRule:PRU00059}.
SimilarityContains 2 Sushi (CCP/SCR) domains. {ECO:0000255|PROSITE-ProRule:PRU00302}.
SubunitC1 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.
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