IED Industry Enzyme Database

Detail Information for IndEnz0002005408
IED ID IndEnz0002005408
Enzyme Type ID protease005408
Protein Name Coagulation factor IX
EC 3.4.21.22
Christmas factor
Plasma thromboplastin component
PTC

Cleaved into: Coagulation factor IXa light chain; Coagulation factor IXa heavy chain
Gene Name F9
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 MQRVNMIMAESPGLITICLLGYLLSAECTVFLDHENANKILNRPKRYNSGKLEEFVQGNLERECMEEKCSFEEAREVFENTERTTEFWKQYVDGDQCESNPCLNGGSCKDDINSYECWCPFGFEGKNCELDVTCNIKNGRCEQFCKNSADNKVVCSCTEGYRLAENQKSCEPAVPFPCGRVSVSQTSKLTRAETVFPDVDYVNSTEAETILDNITQSTQSFNDFTRVVGGEDAKPGQFPWQVVLNGKVDAFCGGSIVNEKWIVTAAHCVETGVKITVVAGEHNIEETEHTEQKRNVIRIIPHHNYNAAINKYNHDIALLELDEPLVLNSYVTPICIADKEYTNIFLKFGSGYVSGWGRVFHKGRSALVLQYLRVPLVDRATCLRSTKFTIYNNMFCAGFHEGGRDSCQGDSGGPHVTEVEGTSFLTGIISWGEECAMKGKYGIYTKVSRYVNWIKEKTKLT
Enzyme Length 461
Uniprot Accession Number P00740
Absorption
Active Site ACT_SITE 267; /note="Charge relay system"; /evidence="ECO:0000269|PubMed:20004170, ECO:0000269|PubMed:659613"; ACT_SITE 315; /note="Charge relay system"; /evidence="ECO:0000269|PubMed:659613"; ACT_SITE 411; /note="Charge relay system"; /evidence="ECO:0000269|PubMed:20004170, ECO:0000269|PubMed:659613"
Activity Regulation
Binding Site
Calcium Binding
catalytic Activity CATALYTIC ACTIVITY: Reaction=Selective cleavage of Arg-|-Ile bond in factor X to form factor Xa.; EC=3.4.21.22; Evidence={ECO:0000269|PubMed:12444082, ECO:0000269|PubMed:20121197, ECO:0000269|PubMed:20121198, ECO:0000269|PubMed:2592373};
DNA Binding
EC Number 3.4.21.22
Enzyme Function FUNCTION: Factor IX is a vitamin K-dependent plasma protein that participates in the intrinsic pathway of blood coagulation by converting factor X to its active form in the presence of Ca(2+) ions, phospholipids, and factor VIIIa. {ECO:0000269|PubMed:1730085, ECO:0000269|PubMed:19846852, ECO:0000269|PubMed:20121197, ECO:0000269|PubMed:20121198, ECO:0000269|PubMed:2592373, ECO:0000269|PubMed:8295821}.
Temperature Dependency
PH Dependency
Pathway
nucleotide Binding
Features Active site (3); Alternative sequence (1); Beta strand (30); Chain (3); Disulfide bond (11); Domain (4); Glycosylation (8); Helix (9); Metal binding (35); Modified residue (17); Mutagenesis (4); Natural variant (156); Propeptide (2); Signal peptide (1); Site (2); Turn (6)
Keywords 3D-structure;Alternative splicing;Blood coagulation;Calcium;Cleavage on pair of basic residues;Direct protein sequencing;Disease variant;Disulfide bond;EGF-like domain;Gamma-carboxyglutamic acid;Glycoprotein;Hemophilia;Hemostasis;Hydrolase;Hydroxylation;Magnesium;Metal-binding;Pharmaceutical;Phosphoprotein;Protease;Reference proteome;Repeat;Secreted;Serine protease;Signal;Sulfation;Thrombophilia;Zymogen
Interact With P00451; Q3U4G3
Induction
Subcellular Location SUBCELLULAR LOCATION: Secreted {ECO:0000269|PubMed:19846852, ECO:0000269|PubMed:2592373, ECO:0000269|PubMed:3857619, ECO:0000269|PubMed:8295821, ECO:0000269|PubMed:9169594}.
Modified Residue MOD_RES 53; /note="4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741, ECO:0000255|PROSITE-ProRule:PRU00463"; MOD_RES 54; /note="4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741, ECO:0000255|PROSITE-ProRule:PRU00463"; MOD_RES 61; /note="4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741, ECO:0000255|PROSITE-ProRule:PRU00463"; MOD_RES 63; /note="4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741, ECO:0000255|PROSITE-ProRule:PRU00463"; MOD_RES 66; /note="4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741, ECO:0000255|PROSITE-ProRule:PRU00463"; MOD_RES 67; /note="4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741, ECO:0000255|PROSITE-ProRule:PRU00463"; MOD_RES 72; /note="4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741, ECO:0000255|PROSITE-ProRule:PRU00463"; MOD_RES 73; /note="4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741, ECO:0000255|PROSITE-ProRule:PRU00463"; MOD_RES 76; /note="4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741, ECO:0000255|PROSITE-ProRule:PRU00463"; MOD_RES 79; /note="4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741, ECO:0000255|PROSITE-ProRule:PRU00463"; MOD_RES 82; /note="4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741, ECO:0000255|PROSITE-ProRule:PRU00463"; MOD_RES 86; /note="4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741, ECO:0000255|PROSITE-ProRule:PRU00463"; MOD_RES 110; /note="(3R)-3-hydroxyaspartate"; /evidence="ECO:0000269|PubMed:6688526"; MOD_RES 114; /note="Phosphoserine"; /evidence="ECO:0000269|Ref.28"; MOD_RES 201; /note="Sulfotyrosine"; /evidence="ECO:0000269|PubMed:11133752"; MOD_RES 204; /note="Phosphoserine"; /evidence="ECO:0000269|PubMed:11133752, ECO:0000269|PubMed:25456591"; MOD_RES 205; /note="Phosphothreonine; alternate"; /evidence="ECO:0000269|PubMed:25456591"
Post Translational Modification PTM: Activated by factor XIa, which excises the activation peptide (PubMed:9169594, PubMed:1730085). The propeptide can also be removed by snake venom protease (PubMed:20004170, PubMed:20080729). {ECO:0000269|PubMed:1730085, ECO:0000269|PubMed:20004170, ECO:0000269|PubMed:20080729, ECO:0000269|PubMed:2592373, ECO:0000269|PubMed:8295821, ECO:0000269|PubMed:9169594}.; PTM: The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains. {ECO:0000269|PubMed:6688526}.
Signal Peptide SIGNAL 1..28; /evidence=ECO:0000255
Structure 3D NMR spectroscopy (4); X-ray crystallography (40)
Cross Reference PDB 1CFH; 1CFI; 1EDM; 1IXA; 1MGX; 1NL0; 1RFN; 2WPH; 2WPI; 2WPJ; 2WPK; 2WPL; 2WPM; 3KCG; 3LC3; 3LC5; 4WM0; 4WMA; 4WMB; 4WMI; 4WMK; 4WN2; 4WNH; 4YZU; 4Z0K; 4ZAE; 5EGM; 5F84; 5F85; 5F86; 5JB8; 5JB9; 5JBA; 5JBB; 5JBC; 5TNO; 5TNT; 5VYG; 6MV4; 6RFK; 6X5J; 6X5L; 6X5P; 7AHV;
Mapped Pubmed ID 10068650; 10468875; 10583536; 10917896; 11122405; 11252894; 11342438; 11396323; 11513608; 11583320; 11754103; 11754413; 11925427; 12036878; 12070021; 12091341; 12105230; 12152682; 12167623; 12192300; 12496253; 12513796; 12522143; 12522212; 12570162; 12768436; 12824704; 12871416; 12871503; 14521595; 14532267; 14567539; 14629468; 14963035; 15009460; 15183040; 15219198; 15326167; 15328360; 15383460; 15456490; 15581349; 15590401; 15634274; 15829482; 15842381; 15866866; 15913649; 15921378; 16102111; 16322469; 16517611; 16766524; 16839351; 16939230; 17048007; 17230038; 17308903; 17397055; 17414217; 17563121; 17882724; 17985356; 18179572; 18192270; 18293119; 18393396; 18434706; 18449417; 18459950; 18600094; 18624698; 18647957; 19047063; 19158249; 19262239; 19286883; 19357501; 19404534; 19416882; 19617576; 19656280; 19699296; 19817987; 19913121; 19930472; 20128871; 20346360; 2040636; 20539913; 20628058; 20628086; 20695909; 20885981; 2105717; 2110473; 21305775; 21976659; 22028648; 22057218; 22207756; 22212890; 22281721; 22471307; 2248955; 22618954; 22639855; 22870602; 22961984; 23014580; 2303434; 23054460; 23093250; 23410583; 23446552; 23472758; 23530052; 23580639; 23585459; 23617568; 23617593; 23884417; 23994528; 24030831; 24068708; 24124147; 24136406; 24261420; 24375831; 24564349; 24656159; 24679056; 24759143; 24816826; 25157807; 25163770; 25224783; 25402191; 25582609; 25851619; 25937013; 25978966; 26023895; 26318999; 26414444; 26612714; 26686734; 26802680; 26928674; 27129300; 27133073; 27208168; 27227676; 27292088; 27356208; 27428513; 2752109; 27529981; 27734074; 27824213; 27865967; 2788012; 28007939; 28196793; 28393470; 28408226; 28729422; 28834196; 29032977; 29223926; 29388273; 29419409; 29656491; 29724133; 29923114; 29993188; 3017409; 30725510; 30817849; 30866119; 31102861; 31180618; 31257730; 31594977; 31677255; 31815744; 32155688; 32346856; 32378017; 32527459; 32678887; 32766856; 32935414; 32996663; 33082527; 33170819; 33397811; 34077951; 34187575; 34412085; 34649996; 34813608; 3667614; 3733688; 3965513; 6334516; 7803238; 7910050; 8148489; 8463288; 8473289; 8530480; 8626656; 9100030; 9427707;
Motif
Gene Encoded By
Mass 51,778
Kinetics
Metal Binding METAL 47; /note="Calcium 1; via carbonyl oxygen"; /evidence="ECO:0000269|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 48; /note="Calcium 2"; /evidence="ECO:0000269|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 53; /note="Calcium 1; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 53; /note="Calcium 2; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 54; /note="Calcium 2; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 54; /note="Calcium 3; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 61; /note="Calcium 4; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 61; /note="Magnesium 1; via 4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741"; METAL 63; /note="Calcium 1; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 63; /note="Calcium 2; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 63; /note="Calcium 3; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 66; /note="Calcium 4; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 66; /note="Magnesium 1; via 4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741"; METAL 67; /note="Calcium 1; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 72; /note="Calcium 5; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 72; /note="Magnesium 2; via 4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741"; METAL 73; /note="Calcium 2; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 73; /note="Calcium 3; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 76; /note="Calcium 3; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 76; /note="Calcium 5; via 4-carboxyglutamate"; /evidence="ECO:0000305|PubMed:14722079, ECO:0007744|PDB:1NL0"; METAL 76; /note="Magnesium 2; via 4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741"; METAL 82; /note="Calcium 6; via 4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741"; METAL 82; /note="Magnesium 3; via 4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741"; METAL 86; /note="Calcium 6; via 4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741"; METAL 86; /note="Magnesium 3; via 4-carboxyglutamate"; /evidence="ECO:0000250|UniProtKB:P00741"; METAL 93; /note="Calcium 7"; /evidence="ECO:0000269|PubMed:7606779, ECO:0007744|PDB:1EDM"; METAL 94; /note="Calcium 7; via carbonyl oxygen"; /evidence="ECO:0000269|PubMed:7606779, ECO:0007744|PDB:1EDM"; METAL 96; /note="Calcium 7"; /evidence="ECO:0000269|PubMed:7606779, ECO:0007744|PDB:1EDM"; METAL 110; /note="Calcium 7"; /evidence="ECO:0000269|PubMed:7606779, ECO:0007744|PDB:1EDM"; METAL 111; /note="Calcium 7; via carbonyl oxygen"; /evidence="ECO:0000269|PubMed:7606779, ECO:0007744|PDB:1EDM"; METAL 281; /note="Calcium 8"; /evidence="ECO:0000269|PubMed:10467148, ECO:0000269|PubMed:20004170, ECO:0000269|PubMed:20080729, ECO:0000269|PubMed:20121197, ECO:0000269|PubMed:20121198, ECO:0007744|PDB:1RFN, ECO:0007744|PDB:2WPH, ECO:0007744|PDB:2WPI, ECO:0007744|PDB:2WPJ, ECO:0007744|PDB:2WPK, ECO:0007744|PDB:2WPM, ECO:0007744|PDB:3KCG, ECO:0007744|PDB:3LC3, ECO:0007744|PDB:3LC5"; METAL 283; /note="Calcium 8; via carbonyl oxygen"; /evidence="ECO:0000269|PubMed:10467148, ECO:0000269|PubMed:20004170, ECO:0000269|PubMed:20080729, ECO:0000269|PubMed:20121197, ECO:0000269|PubMed:20121198, ECO:0007744|PDB:1RFN, ECO:0007744|PDB:2WPH, ECO:0007744|PDB:2WPI, ECO:0007744|PDB:2WPJ, ECO:0007744|PDB:2WPK, ECO:0007744|PDB:2WPM, ECO:0007744|PDB:3KCG, ECO:0007744|PDB:3LC3, ECO:0007744|PDB:3LC5"; METAL 286; /note="Calcium 8; via carbonyl oxygen"; /evidence="ECO:0000269|PubMed:10467148, ECO:0000269|PubMed:20004170, ECO:0000269|PubMed:20080729, ECO:0000269|PubMed:20121197, ECO:0000269|PubMed:20121198, ECO:0007744|PDB:1RFN, ECO:0007744|PDB:2WPH, ECO:0007744|PDB:2WPI, ECO:0007744|PDB:2WPJ, ECO:0007744|PDB:2WPK, ECO:0007744|PDB:2WPM, ECO:0007744|PDB:3KCG, ECO:0007744|PDB:3LC3, ECO:0007744|PDB:3LC5"; METAL 288; /note="Calcium 8"; /evidence="ECO:0000269|PubMed:10467148, ECO:0000269|PubMed:20004170, ECO:0000269|PubMed:20080729, ECO:0000269|PubMed:20121197, ECO:0000269|PubMed:20121198, ECO:0007744|PDB:1RFN, ECO:0007744|PDB:2WPH, ECO:0007744|PDB:2WPI, ECO:0007744|PDB:2WPJ, ECO:0007744|PDB:2WPK, ECO:0007744|PDB:2WPM, ECO:0007744|PDB:3KCG, ECO:0007744|PDB:3LC3, ECO:0007744|PDB:3LC5"; METAL 291; /note="Calcium 8"; /evidence="ECO:0000269|PubMed:10467148, ECO:0000269|PubMed:20004170, ECO:0000269|PubMed:20080729, ECO:0000269|PubMed:20121197, ECO:0000269|PubMed:20121198, ECO:0007744|PDB:2WPH, ECO:0007744|PDB:2WPI, ECO:0007744|PDB:2WPJ, ECO:0007744|PDB:2WPK, ECO:0007744|PDB:2WPM, ECO:0007744|PDB:3KCG, ECO:0007744|PDB:3LC3, ECO:0007744|PDB:3LC5"
Rhea ID
Cross Reference Brenda 3.4.21.22;