IED Industry Enzyme Database

Detail Information for IndEnz0002006051
IED ID IndEnz0002006051
Enzyme Type ID protease006051
Protein Name Coagulation factor VIII
Antihemophilic factor
AHF
Procoagulant component

Cleaved into: Factor VIIIa heavy chain, 200 kDa isoform; Factor VIIIa heavy chain, 92 kDa isoform; Factor VIII B chain; Factor VIIIa light chain
Gene Name F8 F8C
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 MQIELSTCFFLCLLRFCFSATRRYYLGAVELSWDYMQSDLGELPVDARFPPRVPKSFPFNTSVVYKKTLFVEFTDHLFNIAKPRPPWMGLLGPTIQAEVYDTVVITLKNMASHPVSLHAVGVSYWKASEGAEYDDQTSQREKEDDKVFPGGSHTYVWQVLKENGPMASDPLCLTYSYLSHVDLVKDLNSGLIGALLVCREGSLAKEKTQTLHKFILLFAVFDEGKSWHSETKNSLMQDRDAASARAWPKMHTVNGYVNRSLPGLIGCHRKSVYWHVIGMGTTPEVHSIFLEGHTFLVRNHRQASLEISPITFLTAQTLLMDLGQFLLFCHISSHQHDGMEAYVKVDSCPEEPQLRMKNNEEAEDYDDDLTDSEMDVVRFDDDNSPSFIQIRSVAKKHPKTWVHYIAAEEEDWDYAPLVLAPDDRSYKSQYLNNGPQRIGRKYKKVRFMAYTDETFKTREAIQHESGILGPLLYGEVGDTLLIIFKNQASRPYNIYPHGITDVRPLYSRRLPKGVKHLKDFPILPGEIFKYKWTVTVEDGPTKSDPRCLTRYYSSFVNMERDLASGLIGPLLICYKESVDQRGNQIMSDKRNVILFSVFDENRSWYLTENIQRFLPNPAGVQLEDPEFQASNIMHSINGYVFDSLQLSVCLHEVAYWYILSIGAQTDFLSVFFSGYTFKHKMVYEDTLTLFPFSGETVFMSMENPGLWILGCHNSDFRNRGMTALLKVSSCDKNTGDYYEDSYEDISAYLLSKNNAIEPRSFSQNSRHPSTRQKQFNATTIPENDIEKTDPWFAHRTPMPKIQNVSSSDLLMLLRQSPTPHGLSLSDLQEAKYETFSDDPSPGAIDSNNSLSEMTHFRPQLHHSGDMVFTPESGLQLRLNEKLGTTAATELKKLDFKVSSTSNNLISTIPSDNLAAGTDNTSSLGPPSMPVHYDSQLDTTLFGKKSSPLTESGGPLSLSEENNDSKLLESGLMNSQESSWGKNVSSTESGRLFKGKRAHGPALLTKDNALFKVSISLLKTNKTSNNSATNRKTHIDGPSLLIENSPSVWQNILESDTEFKKVTPLIHDRMLMDKNATALRLNHMSNKTTSSKNMEMVQQKKEGPIPPDAQNPDMSFFKMLFLPESARWIQRTHGKNSLNSGQGPSPKQLVSLGPEKSVEGQNFLSEKNKVVVGKGEFTKDVGLKEMVFPSSRNLFLTNLDNLHENNTHNQEKKIQEEIEKKETLIQENVVLPQIHTVTGTKNFMKNLFLLSTRQNVEGSYDGAYAPVLQDFRSLNDSTNRTKKHTAHFSKKGEEENLEGLGNQTKQIVEKYACTTRISPNTSQQNFVTQRSKRALKQFRLPLEETELEKRIIVDDTSTQWSKNMKHLTPSTLTQIDYNEKEKGAITQSPLSDCLTRSHSIPQANRSPLPIAKVSSFPSIRPIYLTRVLFQDNSSHLPAASYRKKDSGVQESSHFLQGAKKNNLSLAILTLEMTGDQREVGSLGTSATNSVTYKKVENTVLPKPDLPKTSGKVELLPKVHIYQKDLFPTETSNGSPGHLDLVEGSLLQGTEGAIKWNEANRPGKVPFLRVATESSAKTPSKLLDPLAWDNHYGTQIPKEEWKSQEKSPEKTAFKKKDTILSLNACESNHAIAAINEGQNKPEIEVTWAKQGRTERLCSQNPPVLKRHQREITRTTLQSDQEEIDYDDTISVEMKKEDFDIYDEDENQSPRSFQKKTRHYFIAAVERLWDYGMSSSPHVLRNRAQSGSVPQFKKVVFQEFTDGSFTQPLYRGELNEHLGLLGPYIRAEVEDNIMVTFRNQASRPYSFYSSLISYEEDQRQGAEPRKNFVKPNETKTYFWKVQHHMAPTKDEFDCKAWAYFSDVDLEKDVHSGLIGPLLVCHTNTLNPAHGRQVTVQEFALFFTIFDETKSWYFTENMERNCRAPCNIQMEDPTFKENYRFHAINGYIMDTLPGLVMAQDQRIRWYLLSMGSNENIHSIHFSGHVFTVRKKEEYKMALYNLYPGVFETVEMLPSKAGIWRVECLIGEHLHAGMSTLFLVYSNKCQTPLGMASGHIRDFQITASGQYGQWAPKLARLHYSGSINAWSTKEPFSWIKVDLLAPMIIHGIKTQGARQKFSSLYISQFIIMYSLDGKKWQTYRGNSTGTLMVFFGNVDSSGIKHNIFNPPIIARYIRLHPTHYSIRSTLRMELMGCDLNSCSMPLGMESKAISDAQITASSYFTNMFATWSPSKARLHLQGRSNAWRPQVNNPKEWLQVDFQKTMKVTGVTTQGVKSLLTSMYVKEFLISSSQDGHQWTLFFQNGKVKVFQGNQDSFTPVVNSLDPPLLTRYLRIHPQSWVHQIALRMEVLGCEAQDLY
Enzyme Length 2351
Uniprot Accession Number P00451
Absorption
Active Site
Activity Regulation
Binding Site
Calcium Binding
catalytic Activity
DNA Binding
EC Number
Enzyme Function FUNCTION: Factor VIII, along with calcium and phospholipid, acts as a cofactor for F9/factor IXa when it converts F10/factor X to the activated form, factor Xa.
Temperature Dependency
PH Dependency
Pathway
nucleotide Binding
Features Alternative sequence (2); Beta strand (11); Chain (5); Disulfide bond (8); Domain (11); Glycosylation (22); Helix (2); Modified residue (6); Natural variant (489); Region (3); Sequence conflict (2); Signal peptide (1); Site (5); Turn (1)
Keywords 3D-structure;Acute phase;Alternative splicing;Blood coagulation;Calcium;Direct protein sequencing;Disease variant;Disulfide bond;Glycoprotein;Hemophilia;Hemostasis;Metal-binding;Pharmaceutical;Reference proteome;Repeat;Secreted;Signal;Sulfation
Interact With P04275; Q8N7X4; Q8N488; P04275; P00740
Induction
Subcellular Location SUBCELLULAR LOCATION: Secreted, extracellular space.
Modified Residue MOD_RES 365; /note="Sulfotyrosine"; /evidence="ECO:0000269|PubMed:10368977, ECO:0000269|PubMed:1554716"; MOD_RES 737; /note="Sulfotyrosine"; /evidence="ECO:0000269|PubMed:10368977"; MOD_RES 738; /note="Sulfotyrosine"; /evidence="ECO:0000269|PubMed:10368977"; MOD_RES 742; /note="Sulfotyrosine"; /evidence="ECO:0000269|PubMed:10368977"; MOD_RES 1683; /note="Sulfotyrosine"; /evidence="ECO:0000269|PubMed:10368977, ECO:0000269|PubMed:1554716"; MOD_RES 1699; /note="Sulfotyrosine"; /evidence="ECO:0000269|PubMed:10368977, ECO:0000269|PubMed:1554716, ECO:0000269|PubMed:1898735"
Post Translational Modification PTM: Sulfation on Tyr-1699 is essential for binding vWF. {ECO:0000269|PubMed:10368977, ECO:0000269|PubMed:1554716, ECO:0000269|PubMed:1898735}.; PTM: Proteolytically cleaved by cathepsin CTSG to produce a partially activated form. {ECO:0000269|PubMed:18217133}.
Signal Peptide SIGNAL 1..19
Structure 3D NMR spectroscopy (2); Electron microscopy (3); X-ray crystallography (13)
Cross Reference PDB 1CFG; 1D7P; 1FAC; 1IQD; 2R7E; 3CDZ; 3HNB; 3HNY; 3HOB; 3J2Q; 3J2S; 4BDV; 4KI5; 4PT6; 4XZU; 5K8D; 6MF2; 7KWO;
Mapped Pubmed ID 10468875; 10586887; 10627467; 10679020; 10747087; 10825291; 10903204; 10982397; 11027931; 11035026; 11057857; 11091190; 11122386; 11128113; 11157485; 11285137; 11306556; 11380445; 11391716; 11396323; 11418455; 11673296; 11722428; 11737010; 11739969; 11769673; 11799130; 11830468; 11848448; 11848452; 11864712; 11927603; 11961312; 12036878; 12041568; 12058364; 12091341; 12154809; 12201837; 12204009; 12220193; 12235121; 12358922; 12368162; 12428094; 12522143; 12567191; 12574801; 12578706; 12615788; 12617176; 12717434; 12719774; 12719775; 12768323; 12857556; 12871600; 12958606; 12969981; 1346975; 14517489; 14521595; 14629468; 14629470; 14644079; 14675089; 14684146; 14706682; 14717992; 14764590; 14961153; 15073030; 15202164; 15213843; 15236327; 15304045; 15353485; 15456493; 15459008; 15471879; 15550021; 15634269; 15660987; 15670040; 15705787; 15710596; 15735794; 15735796; 15824541; 15869621; 15886209; 15913649; 15921397; 15996947; 16038719; 16042406; 16086318; 16088320; 16102111; 16128892; 1618828; 16270627; 16303754; 16339403; 16380445; 16487577; 16513639; 16569771; 16601827; 16601852; 16624868; 16628729; 16732372; 16769589; 16786531; 16823491; 16839343; 16894461; 16894464; 16953269; 16953272; 16956829; 17005010; 17010120; 17038314; 17116206; 17119108; 17192411; 17287628; 17287728; 17316621; 17334514; 17342157; 17349154; 17353931; 17445092; 17498081; 17498085; 17502612; 17610549; 17636198; 17649711; 17650078; 17680530; 17786534; 17823971; 17846507; 17848617; 17849066; 17890957; 17916745; 17927806; 17944985; 17958741; 17965321; 17973851; 18000608; 18000609; 18024377; 18034822; 18064310; 18160577; 18162457; 18166789; 18179574; 18217134; 18217193; 18277139; 18292408; 18299331; 18327416; 18337255; 18371163; 18371166; 18387975; 18388497; 18388498; 18400180; 18403393; 18419741; 18424440; 18435924; 18457169; 18459951; 18479430; 18481121; 18492805; 18495957; 18503540; 18515885; 18521513; 18535282; 18549909; 18600086; 18612535; 18634839; 18647227; 18672380; 18685438; 18702107; 18752533; 18752578; 18803625; 18808737; 18843296; 18983499; 18983511; 1902833; 19080683; 19110358; 19126539; 19132238; 19150994; 19164740; 19187187; 19240027; 19304336; 19329212; 19336741; 19350126; 19369668; 19404520; 19404535; 19473408; 19473423; 19548904; 19563499; 1958666; 19595998; 19624762; 19638414; 19642360; 19656848; 19687511; 19715469; 19719548; 19740093; 19778283; 19778945; 19811836; 19817879; 19825912; 19846852; 19874476; 19913121; 20007547; 20015216; 20028422; 20050928; 20054547; 20062922; 20089867; 20124702; 20153025; 20174619; 20184747; 20193250; 20231535; 20236351; 20237496; 20351306; 20438774; 20456758; 20460344; 20528906; 20533886; 20545907; 20562859; 20586921; 20589311; 20605782; 20606161; 20609463; 20627205; 20628086; 20664893; 20679433; 20682599; 20705334; 20735721; 20735723; 20737444; 20800587; 20966969; 21029358; 21041718; 21044367; 2104832; 21052611; 2105717; 21057709; 21070499; 21071930; 21072517; 21092465; 21094987; 2109644; 2110473; 21143378; 21156843; 21188793; 21210768; 21217077; 21245142; 21305775; 21371201; 21462120; 21470668; 21478858; 21498909; 21532587; 21585650; 21605333; 21606503; 21645180; 21645226; 21668738; 21682900; 21689372; 21705497; 21751985; 21774968; 21810271; 21849206; 21909383; 21909423; 21940821; 21947241; 21953409; 21988832; 21992898; 22013193; 22032085; 22032801; 22057150; 22057218; 22057311; 22103590; 22117735; 22136525; 22157747; 22173226; 22189067; 22219226; 22224589; 22234396; 22270795; 22282501; 22401796; 22422337; 22466813; 22471307; 22505256; 22559004; 22613792; 22621702; 22655063; 22675024; 22776136; 22854959; 22883072; 22906111; 22958177; 22958194; 2303434; 23058466; 23088352; 23111982; 23140572; 23168412; 23178924; 23205847; 23224054; 23238545; 23243272; 23299923; 23306409; 23348756; 23348798; 23349389; 23349392; 23372167; 23378591; 23403259; 23417672; 23435097; 23479643; 23480827; 23481380; 23482934; 23526393; 23534532; 23555096; 23580639; 23616213; 23636243; 23636463; 23640497; 23682841; 23690449; 23696725; 23698585; 23709226; 23754288; 23809411; 23852824; 23884417; 23926300; 23963097; 24009077; 24030831; 24085769; 24108539; 24118514; 24119662; 24167566; 24170271; 24170290; 24211824; 24270469; 24275667; 24296544; 24317041; 24344794; 24497117; 24553346; 24560376; 24578500; 24599523; 24602271; 24668665; 24700780; 24702151; 24814520; 24824129; 24845853; 24917561; 24927536; 24934295; 2502206; 2505252; 25105915; 25157807; 25241761; 25265377; 25315236; 25354263; 25354705; 25382774; 25406352; 25406594; 25503412; 25520269; 25575413; 25586378; 25592075; 25603934; 25605439; 25611311; 25617427; 25619252; 25652415; 25665657; 25669199; 25779970; 25780857; 25824987; 25854144; 25903134; 25948085; 25963793; 26001809; 26023895; 26057490; 26246214; 26264493; 26270389; 26345748; 26346528; 26383047; 26453193; 26473492; 26489971; 26525229; 26598467; 26599105; 26653368; 26743572; 26897466; 26915717; 27013611; 27207787; 27292088; 27455009; 27525858; 27584569; 27587878; 27629384; 27669148; 27734074; 27758819; 27794518; 27824209; 27824213; 27984605; 28056565; 28057741; 28139814; 28327546; 28397397; 28431990; 28432221; 28488800; 28492696; 28492697; 28507083; 28558995; 28683085; 28729433; 28777843; 28799202; 28894750; 29170251; 29357978; 29399993; 29444815; 29534251; 29544691; 29577605; 29614522; 29656491; 29872003; 29890521; 29938987; 30111575; 30642920; 30783064; 3082357; 30901531; 30997536; 31003771; 31063249; 31134694; 3129422; 3134349; 31454152; 31588918; 31675465; 31985870; 31989767; 32032127; 32189311; 32248973; 32453842; 32544272; 32558078; 32589464; 32678887; 32767930; 32791533; 32928254; 32935414; 33082527; 33144078; 33196677; 33221577; 33245802; 33275657; 33342086; 33342805; 33512707; 33569592; 33715149; 34135429; 34177966; 34349063; 34590394; 34662354; 34778454; 3524673; 3965513; 6438527; 6457647; 7727775; 7756647; 7803238; 7910050; 8051097; 8052958; 8144529; 8467233; 8626656; 8663406; 8810344; 8836140; 9094723; 9150144; 9307973; 9427707; 9459313; 9501187; 9601086; 9607108; 9647643; 9774404; 9834200; 9864159; 9920839;
Motif
Gene Encoded By
Mass 267,009
Kinetics
Metal Binding
Rhea ID
Cross Reference Brenda