Complement component 1r

Complement C1r subcomponent (EC 3.4.21.41, activated complement C1r, C overbar 1r esterase, C1r) is a protein involved in the complement system of the innate immune system.^[5][6][7] In humans, C1r is encoded by the C1R gene.^[8]

C1R
Available structures PDB Ortholog search: PDBe RCSB List of PDB id codes 1APQ, 1GPZ, 1MD7, 1MD8, 2QY0
Identifiers
Aliases	C1R, complement C1r, EDSPD1
External IDs	OMIM: 613785; MGI: 3779804; HomoloGene: 1313; GeneCards: C1R; OMA:C1R - orthologs
Gene location (Human) Chr. Chromosome 12 (human)[1] Band 12p13.31 Start 7,080,214 bp[1] End 7,092,540 bp[1]
Gene location (Mouse) Chr. Chromosome 6 (mouse)[2] Band 6 F2|6 Start 124,547,253 bp[2] End 124,558,130 bp[2]
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in right lobe of liver right ovary gallbladder right coronary artery gastric mucosa left ovary left uterine tube left coronary artery urinary bladder canal of the cervix Top expressed in white adipose tissue adrenal gland muscle of thigh urinary bladder dentate gyrus of hippocampal formation granule cell liver ovary skeletal muscle tissue spleen islet of Langerhans More reference expression data BioGPS n/a
Gene ontology Molecular function peptidase activity serine-type peptidase activity serine-type endopeptidase activity protein binding hydrolase activity calcium ion binding Cellular component extracellular region blood microparticle extracellular exosome extracellular space Biological process immune response proteolysis complement activation, classical pathway immune system process innate immune response complement activation regulation of complement activation zymogen activation Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 715 667277 Ensembl ENSG00000159403 ENSG00000288512 ENSMUSG00000098470 UniProt P00736 Q8CFG9 RefSeq (mRNA) NM_001733 NM_001354346 NM_001113356 RefSeq (protein) NP_001724 NP_001341275 NP_001106827 Location (UCSC) Chr 12: 7.08 – 7.09 Mb Chr 6: 124.55 – 124.56 Mb PubMed search [3] [4]
Wikidata
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C1r along with C1q and C1s form the C1 complex, which is the first component of the serum complement system. C1r is an enzyme that activates C1s to its active form, by proteolytic cleavage.

Clinical significance

• Ehlers–Danlos syndrome Periodontal type is associated with mutations in the CR1 gene

Function

C1r has been shown to interact with C1s. C1r cleaves C1s to form the active form of C1s.^[9][10]