| IED ID | IndEnz0002014131 |
| Enzyme Type ID | protease014131 |
| Protein Name |
Complement C1r subcomponent EC 3.4.21.41 Complement component 1 subcomponent r Cleaved into: Complement C1r subcomponent heavy chain; Complement C1r subcomponent light chain |
| Gene Name | C1R |
| Organism | Homo sapiens (Human) |
| Taxonomic Lineage | cellular organisms Eukaryota Opisthokonta Metazoa Eumetazoa Bilateria Deuterostomia Chordata Craniata Vertebrata Gnathostomata (jawed vertebrates) Teleostomi Euteleostomi Sarcopterygii Dipnotetrapodomorpha Tetrapoda Amniota Mammalia Theria Eutheria Boreoeutheria Euarchontoglires Primates Haplorrhini Simiiformes Catarrhini Hominoidea (apes) Hominidae (great apes) Homininae Homo Homo sapiens (Human) |
| Enzyme Sequence | MWLLYLLVPALFCRAGGSIPIPQKLFGEVTSPLFPKPYPNNFETTTVITVPTGYRVKLVFQQFDLEPSEGCFYDYVKISADKKSLGRFCGQLGSPLGNPPGKKEFMSQGNKMLLTFHTDFSNEENGTIMFYKGFLAYYQAVDLDECASRSKSGEEDPQPQCQHLCHNYVGGYFCSCRPGYELQEDTHSCQAECSSELYTEASGYISSLEYPRSYPPDLRCNYSIRVERGLTLHLKFLEPFDIDDHQQVHCPYDQLQIYANGKNIGEFCGKQRPPDLDTSSNAVDLLFFTDESGDSRGWKLRYTTEIIKCPQPKTLDEFTIIQNLQPQYQFRDYFIATCKQGYQLIEGNQVLHSFTAVCQDDGTWHRAMPRCKIKDCGQPRNLPNGDFRYTTTMGVNTYKARIQYYCHEPYYKMQTRAGSRESEQGVYTCTAQGIWKNEQKGEKIPRCLPVCGKPVNPVEQRQRIIGGQKAKMGNFPWQVFTNIHGRGGGALLGDRWILTAAHTLYPKEHEAQSNASLDVFLGHTNVEELMKLGNHPIRRVSVHPDYRQDESYNFEGDIALLELENSVTLGPNLLPICLPDNDTFYDLGLMGYVSGFGVMEEKIAHDLRFVRLPVANPQACENWLRGKNRMDVFSQNMFCAGHPSLKQDACQGDSGGVFAVRDPNTDRWVATGIVSWGIGCSRGYGFYTKVLNYVDWIKKEMEEED |
| Enzyme Length | 705 |
| Uniprot Accession Number | P00736 |
| Absorption | |
| Active Site | ACT_SITE 502; /note=Charge relay system; ACT_SITE 557; /note=Charge relay system; ACT_SITE 654; /note=Charge relay system |
| Activity Regulation | |
| Binding Site | |
| Calcium Binding | |
| catalytic Activity | CATALYTIC ACTIVITY: Reaction=Selective cleavage of Lys(or Arg)-|-Ile bond in complement subcomponent C1s to form the active form of C1s (EC 3.4.21.42).; EC=3.4.21.41; |
| DNA Binding | |
| EC Number | 3.4.21.41 |
| Enzyme Function | 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. |
| Temperature Dependency | |
| PH Dependency | |
| Pathway | |
| nucleotide Binding | |
| Features | Active site (3); Beta strand (42); Chain (3); Disulfide bond (13); Domain (6); Glycosylation (4); Helix (7); Modified residue (2); Natural variant (20); Signal peptide (1); Turn (8) |
| Keywords | 3D-structure;Complement pathway;Direct protein sequencing;Disease variant;Disulfide bond;EGF-like domain;Ehlers-Danlos syndrome;Glycoprotein;Hydrolase;Hydroxylation;Immunity;Innate immunity;Phosphoprotein;Protease;Reference proteome;Repeat;Secreted;Serine protease;Signal;Sushi |
| Interact With | Itself; P09871 |
| Induction | |
| Subcellular Location | SUBCELLULAR LOCATION: Secreted {ECO:0000269|PubMed:27745832}. |
| Modified Residue | MOD_RES 167; /note=(3R)-3-hydroxyasparagine; /evidence=ECO:0000269|PubMed:2820791; MOD_RES 206; /note=Phosphoserine; by CK2; /evidence=ECO:0000269|PubMed:8635594 |
| Post Translational Modification | PTM: The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains. {ECO:0000269|PubMed:2820791}. |
| Signal Peptide | SIGNAL 1..17; /evidence=ECO:0000269|PubMed:3036070 |
| Structure 3D | NMR spectroscopy (1); X-ray crystallography (8) |
| Cross Reference PDB | 1APQ; 1GPZ; 1MD7; 1MD8; 2QY0; 6F1C; 6F1D; 6F1H; 6F39; |
| Mapped Pubmed ID | 10925294; 11445589; 12396008; 1249422; 12499050; 12555245; 12788922; 14280442; 15199963; 17996945; 20008834; 20178990; 20592021; 20796027; 20970424; 21988832; 23300094; 23589288; 23650384; 2387866; 26231209; 26709396; 28104818; 28544690; 29311313; 30535813; 31749804; 34155115; 6019133; 70787; 760802; 761607; 814163; |
| Motif | |
| Gene Encoded By | |
| Mass | 80,119 |
| Kinetics | |
| Metal Binding | |
| Rhea ID | |
| Cross Reference Brenda | 3.4.21.41; |