Names & Taxonomy

Uniprot ID:
P00451
Entry Name:
FA8_HUMAN
Status:
reviewed
Protein Names:
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 Names:
F8 F8C
Gene Names Primary:
F8
Organism:
Homo sapiens (Human)

Structure

Length:
2351
Sequence:
MQIELSTCFFLCLLRFCFSATRRYYLGAVELSWDYMQSDLGELPVDARFPPRVPKSFPFNTSVVYKKTLFVEFTDHLFNIAKPRPPWMGLLGPTIQAEVYDTVVITLKNMASHPVSLHAVGVSYWKASEGAEYDDQTSQREKEDDKVFPGGSHTYVWQVLKENGPMASDPLCLTYSYLSHVDLVKDLNSGLIGALLVCREGSLAKEKTQTLHKFILLFAVFDEGKSWHSETKNSLMQDRDAASARAWPKMHTVNGYVNRSLPGLIGCHRKSVYWHVIGMGTTPEVHSIFLEGHTFLVRNHRQASLEISPITFLTAQTLLMDLGQFLLFCHISSHQHDGMEAYVKVDSCPEEPQLRMKNNEEAEDYDDDLTDSEMDVVRFDDDNSPSFIQIRSVAKKHPKTWVHYIAAEEEDWDYAPLVLAPDDRSYKSQYLNNGPQRIGRKYKKVRFMAYTDETFKTREAIQHESGILGPLLYGEVGDTLLIIFKNQASRPYNIYPHGITDVRPLYSRRLPKGVKHLKDFPILPGEIFKYKWTVTVEDGPTKSDPRCLTRYYSSFVNMERDLASGLIGPLLICYKESVDQRGNQIMSDKRNVILFSVFDENRSWYLTENIQRFLPNPAGVQLEDPEFQASNIMHSINGYVFDSLQLSVCLHEVAYWYILSIGAQTDFLSVFFSGYTFKHKMVYEDTLTLFPFSGETVFMSMENPGLWILGCHNSDFRNRGMTALLKVSSCDKNTGDYYEDSYEDISAYLLSKNNAIEPRSFSQNSRHPSTRQKQFNATTIPENDIEKTDPWFAHRTPMPKIQNVSSSDLLMLLRQSPTPHGLSLSDLQEAKYETFSDDPSPGAIDSNNSLSEMTHFRPQLHHSGDMVFTPESGLQLRLNEKLGTTAATELKKLDFKVSSTSNNLISTIPSDNLAAGTDNTSSLGPPSMPVHYDSQLDTTLFGKKSSPLTESGGPLSLSEENNDSKLLESGLMNSQESSWGKNVSSTESGRLFKGKRAHGPALLTKDNALFKVSISLLKTNKTSNNSATNRKTHIDGPSLLIENSPSVWQNILESDTEFKKVTPLIHDRMLMDKNATALRLNHMSNKTTSSKNMEMVQQKKEGPIPPDAQNPDMSFFKMLFLPESARWIQRTHGKNSLNSGQGPSPKQLVSLGPEKSVEGQNFLSEKNKVVVGKGEFTKDVGLKEMVFPSSRNLFLTNLDNLHENNTHNQEKKIQEEIEKKETLIQENVVLPQIHTVTGTKNFMKNLFLLSTRQNVEGSYDGAYAPVLQDFRSLNDSTNRTKKHTAHFSKKGEEENLEGLGNQTKQIVEKYACTTRISPNTSQQNFVTQRSKRALKQFRLPLEETELEKRIIVDDTSTQWSKNMKHLTPSTLTQIDYNEKEKGAITQSPLSDCLTRSHSIPQANRSPLPIAKVSSFPSIRPIYLTRVLFQDNSSHLPAASYRKKDSGVQESSHFLQGAKKNNLSLAILTLEMTGDQREVGSLGTSATNSVTYKKVENTVLPKPDLPKTSGKVELLPKVHIYQKDLFPTETSNGSPGHLDLVEGSLLQGTEGAIKWNEANRPGKVPFLRVATESSAKTPSKLLDPLAWDNHYGTQIPKEEWKSQEKSPEKTAFKKKDTILSLNACESNHAIAAINEGQNKPEIEVTWAKQGRTERLCSQNPPVLKRHQREITRTTLQSDQEEIDYDDTISVEMKKEDFDIYDEDENQSPRSFQKKTRHYFIAAVERLWDYGMSSSPHVLRNRAQSGSVPQFKKVVFQEFTDGSFTQPLYRGELNEHLGLLGPYIRAEVEDNIMVTFRNQASRPYSFYSSLISYEEDQRQGAEPRKNFVKPNETKTYFWKVQHHMAPTKDEFDCKAWAYFSDVDLEKDVHSGLIGPLLVCHTNTLNPAHGRQVTVQEFALFFTIFDETKSWYFTENMERNCRAPCNIQMEDPTFKENYRFHAINGYIMDTLPGLVMAQDQRIRWYLLSMGSNENIHSIHFSGHVFTVRKKEEYKMALYNLYPGVFETVEMLPSKAGIWRVECLIGEHLHAGMSTLFLVYSNKCQTPLGMASGHIRDFQITASGQYGQWAPKLARLHYSGSINAWSTKEPFSWIKVDLLAPMIIHGIKTQGARQKFSSLYISQFIIMYSLDGKKWQTYRGNSTGTLMVFFGNVDSSGIKHNIFNPPIIARYIRLHPTHYSIRSTLRMELMGCDLNSCSMPLGMESKAISDAQITASSYFTNMFATWSPSKARLHLQGRSNAWRPQVNNPKEWLQVDFQKTMKVTGVTTQGVKSLLTSMYVKEFLISSSQDGHQWTLFFQNGKVKVFQGNQDSFTPVVNSLDPPLLTRYLRIHPQSWVHQIALRMEVLGCEAQDLY
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Secreted, extracellular space.

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.
Cross Reference Drug Bank:
DB00100 DB00055
Gene Ontology Go:
endoplasmic reticulum lumen
endoplasmic reticulum-Golgi intermediate compartment membrane
ER to Golgi transport vesicle
extracellular region
extracellular space
Golgi membrane
plasma membrane
platelet alpha granule lumen
copper ion binding
oxidoreductase activity
acute-phase response
blood coagulation
blood coagulation, intrinsic pathway
cellular protein metabolic process
COPII vesicle coating
ER to Golgi vesicle-mediated transport
membrane organization
platelet activation
platelet degranulation
post-translational protein modification
protein N-linked glycosylation via asparagine
Gene Ontology Biological Process:
acute-phase response
blood coagulation
blood coagulation, intrinsic pathway
cellular protein metabolic process
COPII vesicle coating
ER to Golgi vesicle-mediated transport
membrane organization
platelet activation
platelet degranulation
post-translational protein modification
protein N-linked glycosylation via asparagine
Gene Ontology Molecular Function:
copper ion binding
oxidoreductase activity
Gene Ontology Cellular Component:
endoplasmic reticulum-Golgi intermediate compartment membrane
endoplasmic reticulum lumen
ER to Golgi transport vesicle
extracellular region
extracellular space
Golgi membrane
plasma membrane
platelet alpha granule lumen
Keywords:
3D-structure
Acute phase
Alternative splicing
Blood coagulation
Calcium
Complete proteome
Direct protein sequencing
Disease mutation
Disulfide bond
Glycoprotein
Hemophilia
Hemostasis
Metal-binding
Pharmaceutical
Polymorphism
Reference proteome
Repeat
Secreted
Signal
Sulfation

Publication

PubMed ID:
3935400 6438526 1427887 6438528 1303178 14702039 15772651 15489334 10368977 1898735 1554716 9218428 7613471 16335952 7893714 1902642 2491949 7728145 3012775 3122181 2833855 2835904 2499363 2506948 2510835 2495245 2498882 2104766 2105106 1973901 2105906 2106480 2107542 1908817 1908096 1924291 1851341 1356412 1639429 1349567 1301194 1301932 1301960 8449505 8322269 7579394 7794769 7759074 8644728 8639447 8759905 9029040 9326186 9341862 9886318 9450898 10215414 9603440 9452104 9792405 9829908 9569180 9569189 10554831 10338101 10408784 10404764 10910910 10910913 10691849 10886198 10800171 10896236 10612839 11410838 11298607 11442643 11442647 11554935 11748850 11341489 12351418 12406074 12199686 11857744 12203998 12325022 11858487 12195713 12930394 12871415 12614369 15682412 15810915 16805874 18184865 21371196 25550078 26278069