P00451: Coagulation factor VIII

General Information

Protein names	- Coagulation factor VIII - Antihemophilic factor - AHF - Procoagulant component - Factor VIIIa heavy chain, 200 kDa isoform - Factor VIIIa heavy chain, 92 kDa isoform - Factor VIII B chain - Factor VIIIa light chain
Gene names	F8
Organism	Homo sapiens
Protease Family
Protease ID
Chromosome location
UniProt ID	P00451

9 N-termini

9 C-termini

18 Cleavages

0 Substrates

Sequence

        10         20         30         40         50         60
MQIELSTCFF LCLLRFCFSA TRRYYLGAVE LSWDYMQSDL GELPVDARFP PRVPKSFPFN
        70         80         90        100        110        120
TSVVYKKTLF VEFTDHLFNI AKPRPPWMGL LGPTIQAEVY DTVVITLKNM ASHPVSLHAV
       130        140        150        160        170        180
GVSYWKASEG AEYDDQTSQR EKEDDKVFPG GSHTYVWQVL KENGPMASDP LCLTYSYLSH
       190        200        210        220        230        240
VDLVKDLNSG LIGALLVCRE GSLAKEKTQT LHKFILLFAV FDEGKSWHSE TKNSLMQDRD
       250        260        270        280        290        300
AASARAWPKM HTVNGYVNRS LPGLIGCHRK SVYWHVIGMG TTPEVHSIFL EGHTFLVRNH
       310        320        330        340        350        360
RQASLEISPI TFLTAQTLLM DLGQFLLFCH ISSHQHDGME AYVKVDSCPE EPQLRMKNNE
       370        380        390        400        410        420
EAEDYDDDLT DSEMDVVRFD DDNSPSFIQI RSVAKKHPKT WVHYIAAEEE DWDYAPLVLA
       430        440        450        460        470        480
PDDRSYKSQY LNNGPQRIGR KYKKVRFMAY TDETFKTREA IQHESGILGP LLYGEVGDTL
       490        500        510        520        530        540
LIIFKNQASR PYNIYPHGIT DVRPLYSRRL PKGVKHLKDF PILPGEIFKY KWTVTVEDGP
       550        560        570        580        590        600
TKSDPRCLTR YYSSFVNMER DLASGLIGPL LICYKESVDQ RGNQIMSDKR NVILFSVFDE
       610        620        630        640        650        660
NRSWYLTENI QRFLPNPAGV QLEDPEFQAS NIMHSINGYV FDSLQLSVCL HEVAYWYILS
       670        680        690        700        710        720
IGAQTDFLSV FFSGYTFKHK MVYEDTLTLF PFSGETVFMS MENPGLWILG CHNSDFRNRG
       730        740        750        760        770        780
MTALLKVSSC DKNTGDYYED SYEDISAYLL SKNNAIEPRS FSQNSRHPST RQKQFNATTI
       790        800        810        820        830        840
PENDIEKTDP WFAHRTPMPK IQNVSSSDLL MLLRQSPTPH GLSLSDLQEA KYETFSDDPS
       850        860        870        880        890        900
PGAIDSNNSL SEMTHFRPQL HHSGDMVFTP ESGLQLRLNE KLGTTAATEL KKLDFKVSST
       910        920        930        940        950        960
SNNLISTIPS DNLAAGTDNT SSLGPPSMPV HYDSQLDTTL FGKKSSPLTE SGGPLSLSEE
       970        980        990       1000       1010       1020
NNDSKLLESG LMNSQESSWG KNVSSTESGR LFKGKRAHGP ALLTKDNALF KVSISLLKTN
      1030       1040       1050       1060       1070       1080
KTSNNSATNR KTHIDGPSLL IENSPSVWQN ILESDTEFKK VTPLIHDRML MDKNATALRL
      1090       1100       1110       1120       1130       1140
NHMSNKTTSS KNMEMVQQKK EGPIPPDAQN PDMSFFKMLF LPESARWIQR THGKNSLNSG
      1150       1160       1170       1180       1190       1200
QGPSPKQLVS LGPEKSVEGQ NFLSEKNKVV VGKGEFTKDV GLKEMVFPSS RNLFLTNLDN
      1210       1220       1230       1240       1250       1260
LHENNTHNQE KKIQEEIEKK ETLIQENVVL PQIHTVTGTK NFMKNLFLLS TRQNVEGSYD
      1270       1280       1290       1300       1310       1320
GAYAPVLQDF RSLNDSTNRT KKHTAHFSKK GEEENLEGLG NQTKQIVEKY ACTTRISPNT
      1330       1340       1350       1360       1370       1380
SQQNFVTQRS KRALKQFRLP LEETELEKRI IVDDTSTQWS KNMKHLTPST LTQIDYNEKE
      1390       1400       1410       1420       1430       1440
KGAITQSPLS DCLTRSHSIP QANRSPLPIA KVSSFPSIRP IYLTRVLFQD NSSHLPAASY
      1450       1460       1470       1480       1490       1500
RKKDSGVQES SHFLQGAKKN NLSLAILTLE MTGDQREVGS LGTSATNSVT YKKVENTVLP
      1510       1520       1530       1540       1550       1560
KPDLPKTSGK VELLPKVHIY QKDLFPTETS NGSPGHLDLV EGSLLQGTEG AIKWNEANRP
      1570       1580       1590       1600       1610       1620
GKVPFLRVAT ESSAKTPSKL LDPLAWDNHY GTQIPKEEWK SQEKSPEKTA FKKKDTILSL
      1630       1640       1650       1660       1670       1680
NACESNHAIA AINEGQNKPE IEVTWAKQGR TERLCSQNPP VLKRHQREIT RTTLQSDQEE
      1690       1700       1710       1720       1730       1740
IDYDDTISVE MKKEDFDIYD EDENQSPRSF QKKTRHYFIA AVERLWDYGM SSSPHVLRNR
      1750       1760       1770       1780       1790       1800
AQSGSVPQFK KVVFQEFTDG SFTQPLYRGE LNEHLGLLGP YIRAEVEDNI MVTFRNQASR
      1810       1820       1830       1840       1850       1860
PYSFYSSLIS YEEDQRQGAE PRKNFVKPNE TKTYFWKVQH HMAPTKDEFD CKAWAYFSDV
      1870       1880       1890       1900       1910       1920
DLEKDVHSGL IGPLLVCHTN TLNPAHGRQV TVQEFALFFT IFDETKSWYF TENMERNCRA
      1930       1940       1950       1960       1970       1980
PCNIQMEDPT FKENYRFHAI NGYIMDTLPG LVMAQDQRIR WYLLSMGSNE NIHSIHFSGH
      1990       2000       2010       2020       2030       2040
VFTVRKKEEY KMALYNLYPG VFETVEMLPS KAGIWRVECL IGEHLHAGMS TLFLVYSNKC
      2050       2060       2070       2080       2090       2100
QTPLGMASGH IRDFQITASG QYGQWAPKLA RLHYSGSINA WSTKEPFSWI KVDLLAPMII
      2110       2120       2130       2140       2150       2160
HGIKTQGARQ KFSSLYISQF IIMYSLDGKK WQTYRGNSTG TLMVFFGNVD SSGIKHNIFN
      2170       2180       2190       2200       2210       2220
PPIIARYIRL HPTHYSIRST LRMELMGCDL NSCSMPLGME SKAISDAQIT ASSYFTNMFA
      2230       2240       2250       2260       2270       2280
TWSPSKARLH LQGRSNAWRP QVNNPKEWLQ VDFQKTMKVT GVTTQGVKSL LTSMYVKEFL
      2290       2300       2310       2320       2330       2340
ISSSQDGHQW TLFFQNGKVK VFQGNQDSFT PVVNSLDPPL LTRYLRIHPQ SWVHQIALRM
      2350
EVLGCEAQDL Y

Isoforms

- Isoform 2 of Coagulation factor VIII

Sequence View

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Protein Neighborhood

Domains & Features

9 N-termini - 9 C-termini - 18 Cleavages - 0 Substrates

N-termini

Name	Sequence	Position	Evidence type	Method	Source (database)	Evidence name
P00451-20-unknown	ATRRYY...	20	inferred from electronic annotation	electronic annotation	UniProtKB	inferred from uniprot
P00451-56-unknown	SFPFNT...	56	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27433
P00451-56-unknown	SFPFNT...	56	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC9590
P00451-356-unknown	MKNNEE...	356	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27434
P00451-356-unknown	MKNNEE...	356	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27760
P00451-356-unknown	MKNNEE...	356	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC9591
P00451-356-unknown	MKNNEE...	356	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC9644
P00451-392-unknown	SVAKKH...	392	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27435
P00451-392-unknown	SVAKKH...	392	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC9592
P00451-392-unknown	SVAKKH...	392	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC31719
P00451-582-unknown	GNQIMS...	582	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27761
P00451-760-unknown	SFSQNS...	760	inferred from electronic annotation	electronic annotation	UniProtKB	inferred from uniprot
P00451-760-unknown	SFSQNS...	760	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27436
P00451-760-unknown	SFSQNS...	760	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27762
P00451-760-unknown	SFSQNS...	760	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC9593
P00451-760-unknown	SFSQNS...	760	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC31720
P00451-1668-unknown	EITRTT...	1668	inferred from electronic annotation	electronic annotation	UniProtKB	inferred from uniprot
P00451-1709-unknown	SFQKKT...	1709	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27437
P00451-1709-unknown	SFQKKT...	1709	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC9594
P00451-1709-unknown	SFQKKT...	1709	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC31721
P00451-1741-unknown	AQSGSV...	1741	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27438

C-termini

Sequence	Position	Evidence type	Method	Source (database)	Evidence name
...PRVPKS	55	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27433
...PRVPKS	55	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC9590
...EPQLRM	355	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27434
...EPQLRM	355	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27760
...EPQLRM	355	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC9591
...EPQLRM	355	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC9644
...FIQIRS	391	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27435
...FIQIRS	391	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC9592
...FIQIRS	391	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC31719
...SVDQRG	581	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27761
...AIEPRS	759	inferred from electronic annotation	electronic annotation	UniProtKB	inferred from uniprot
...AIEPRS	759	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27436
...AIEPRS	759	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27762
...AIEPRS	759	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC9593
...AIEPRS	759	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC31720
...QRSKRA	1332	inferred from electronic annotation	electronic annotation	UniProtKB	inferred from uniprot
...NQSPRS	1708	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27437
...NQSPRS	1708	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC9594
...NQSPRS	1708	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC31721
...VLRNRA	1740	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27438
...AQDLY	2351	inferred from electronic annotation	electronic annotation	UniProtKB	inferred from uniprot
...AQDLY	2351	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TCt70320

Cleavages

Protease	Position	Sequence	Evidence type	Method	Source (database)	Source (Lab)	Evidence name	Publications (PMIDs)
PLMN_HUMAN	55	VPKS.\|.SFPF	inferred from experiment	unknown	MEROPS	Yoshioka A	Nogami K et al.:Mechanisms of plasmin-catalyzed... (S01.233)	17189254,
FA10_HUMAN	55	VPKS.\|.SFPF	inferred from experiment	unknown	MEROPS	Fay PJ	Nogami K et al.:Mechanisms of factor Xa-catalyz... (S01.216)	12606556,
PLMN_HUMAN	355	QLRM.\|.MKNN	inferred from experiment	unknown	MEROPS	Yoshioka A	Nogami K et al.:Mechanisms of plasmin-catalyzed... (S01.233)	17189254,
PROC_HUMAN	355	QLRM.\|.MKNN	inferred from experiment	unknown	MEROPS	Fay PJ	Nogami K et al.:Mechanisms of factor Xa-catalyz... (S01.216)	12606556,
FA10_HUMAN	355	QLRM.\|.MKNN	inferred from experiment	unknown	MEROPS	Fay PJ	Nogami K et al.:Mechanisms of factor Xa-catalyz... (S01.216)	12606556,
FA9_HUMAN	355	QLRM.\|.MKNN	inferred from experiment	unknown	MEROPS	Fay PJ	Nogami K et al.:Mechanisms of factor Xa-catalyz... (S01.216)	12606556,
PLMN_HUMAN	391	QIRS.\|.SVAK	inferred from experiment	unknown	MEROPS	Yoshioka A	Nogami K et al.:Mechanisms of plasmin-catalyzed... (S01.233)	17189254,
FA10_HUMAN	391	QIRS.\|.SVAK	inferred from experiment	unknown	MEROPS	Fay PJ	Nogami K et al.:Mechanisms of factor Xa-catalyz... (S01.216)	12606556,
THRB_HUMAN	391	QIRS.\|.SVAK	inferred from experiment	unknown	MEROPS	Neuenschwander PF	Warren DL et al.:Proteolysis of blood coagulatio... (S01.217)	10350471,
PROC_HUMAN	581	DQRG.\|.GNQI	inferred from experiment	unknown	MEROPS	Walker FJ	Fay PJ et al.:Activated protein C-catalyzed i... (S01.218)	1939075,
PLMN_HUMAN	759	EPRS.\|.SFSQ	inferred from experiment	unknown	MEROPS	Yoshioka A	Nogami K et al.:Mechanisms of plasmin-catalyzed... (S01.233)	17189254,
PROC_HUMAN	759	EPRS.\|.SFSQ	inferred from experiment	unknown	MEROPS	Walker FJ	Fay PJ et al.:Activated protein C-catalyzed i... (S01.218)	1939075,
FA10_HUMAN	759	EPRS.\|.SFSQ	inferred from experiment	unknown	MEROPS	Fay PJ	Nogami K et al.:Mechanisms of factor Xa-catalyz... (S01.216)	12606556,
THRB_HUMAN	759	EPRS.\|.SFSQ	inferred from experiment	unknown	MEROPS	Neuenschwander PF	Warren DL et al.:Proteolysis of blood coagulatio... (S01.217)	10350471,
PLMN_HUMAN	1708	SPRS.\|.SFQK	inferred from experiment	unknown	MEROPS	Yoshioka A	Nogami K et al.:Mechanisms of plasmin-catalyzed... (S01.233)	17189254,
FA10_HUMAN	1708	SPRS.\|.SFQK	inferred from experiment	unknown	MEROPS	Fay PJ	Nogami K et al.:Mechanisms of factor Xa-catalyz... (S01.216)	12606556,
THRB_HUMAN	1708	SPRS.\|.SFQK	inferred from experiment	unknown	MEROPS	Neuenschwander PF	Warren DL et al.:Proteolysis of blood coagulatio... (S01.217)	10350471,
PLMN_HUMAN	1740	RNRA.\|.AQSG	inferred from experiment	unknown	MEROPS	Yoshioka A	Nogami K et al.:Mechanisms of plasmin-catalyzed... (S01.233)	17189254,

Substrates

Substrate	Position	Sequence	Evidence type	Method	Source (database)	Source (Lab)	Evidence name	Publications (PMIDs)