Result

  Coagulation factor VIII

Source	Homo sapiens (human)
Taxonomy	Homo sapiens Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo.
Keywords	3D-structure; Acute phase; Blood coagulation; Calcium; Complete proteome; Direct protein sequencing; Disease mutation; Disulfide bond; Glycoprotein; Hemophilia; Metal-binding; Pharmaceutical; Polymorphism; Repeat; Secreted; Signal; Sulfation.
Details Function: Factor VIII, along with calcium and phospholipid, acts as a cofactor for factor IXa when it converts factor X to the activated form, factor Xa. Post-translational modification: Sulfation on Tyr-1699 is essential for binding vWF. Similarity: Belongs to the multicopper oxidase family. Contains 3 F5/8 type A domains. Contains 2 F5/8 type C domains. Contains 6 plastocyanin-like domains. Subcellular location: Secreted, extracellular space. Subunit structure: Interacts with vWF. vWF binding is essential for the stabilization of F8 in circulation. Disease: Defects in F8 are the cause of hemophilia A (HEMA) Sequence length: 2351 AA.
Sequence MQIELSTCFFLCLLRFCFSATRRYYLGAVELSWDYMQSDLGELPVDARFPPRVPKSFPFN TSVVYKKTLFVEFTDHLFNIAKPRPPWMGLLGPTIQAEVYDTVVITLKNMASHPVSLHAV GVSYWKASEGAEYDDQTSQREKEDDKVFPGGSHTYVWQVLKENGPMASDPLCLTYSYLSH VDLVKDLNSGLIGALLVCREGSLAKEKTQTLHKFILLFAVFDEGKSWHSETKNSLMQDRD AASARAWPKMHTVNGYVNRSLPGLIGCHRKSVYWHVIGMGTTPEVHSIFLEGHTFLVRNH RQASLEISPITFLTAQTLLMDLGQFLLFCHISSHQHDGMEAYVKVDSCPEEPQLRMKNNE EAEDYDDDLTDSEMDVVRFDDDNSPSFIQIRSVAKKHPKTWVHYIAAEEEDWDYAPLVLA PDDRSYKSQYLNNGPQRIGRKYKKVRFMAYTDETFKTREAIQHESGILGPLLYGEVGDTL LIIFKNQASRPYNIYPHGITDVRPLYSRRLPKGVKHLKDFPILPGEIFKYKWTVTVEDGP TKSDPRCLTRYYSSFVNMERDLASGLIGPLLICYKESVDQRGNQIMSDKRNVILFSVFDE NRSWYLTENIQRFLPNPAGVQLEDPEFQASNIMHSINGYVFDSLQLSVCLHEVAYWYILS IGAQTDFLSVFFSGYTFKHKMVYEDTLTLFPFSGETVFMSMENPGLWILGCHNSDFRNRG MTALLKVSSCDKNTGDYYEDSYEDISAYLLSKNNAIEPRSFSQNSRHPSTRQKQFNATTI PENDIEKTDPWFAHRTPMPKIQNVSSSDLLMLLRQSPTPHGLSLSDLQEAKYETFSDDPS PGAIDSNNSLSEMTHFRPQLHHSGDMVFTPESGLQLRLNEKLGTTAATELKKLDFKVSST SNNLISTIPSDNLAAGTDNTSSLGPPSMPVHYDSQLDTTLFGKKSSPLTESGGPLSLSEE NNDSKLLESGLMNSQESSWGKNVSSTESGRLFKGKRAHGPALLTKDNALFKVSISLLKTN KTSNNSATNRKTHIDGPSLLIENSPSVWQNILESDTEFKKVTPLIHDRMLMDKNATALRL NHMSNKTTSSKNMEMVQQKKEGPIPPDAQNPDMSFFKMLFLPESARWIQRTHGKNSLNSG QGPSPKQLVSLGPEKSVEGQNFLSEKNKVVVGKGEFTKDVGLKEMVFPSSRNLFLTNLDN LHENNTHNQEKKIQEEIEKKETLIQENVVLPQIHTVTGTKNFMKNLFLLSTRQNVEGSYD GAYAPVLQDFRSLNDSTNRTKKHTAHFSKKGEEENLEGLGNQTKQIVEKYACTTRISPNT SQQNFVTQRSKRALKQFRLPLEETELEKRIIVDDTSTQWSKNMKHLTPSTLTQIDYNEKE KGAITQSPLSDCLTRSHSIPQANRSPLPIAKVSSFPSIRPIYLTRVLFQDNSSHLPAASY RKKDSGVQESSHFLQGAKKNNLSLAILTLEMTGDQREVGSLGTSATNSVTYKKVENTVLP KPDLPKTSGKVELLPKVHIYQKDLFPTETSNGSPGHLDLVEGSLLQGTEGAIKWNEANRP GKVPFLRVATESSAKTPSKLLDPLAWDNHYGTQIPKEEWKSQEKSPEKTAFKKKDTILSL NACESNHAIAAINEGQNKPEIEVTWAKQGRTERLCSQNPPVLKRHQREITRTTLQSDQEE IDYDDTISVEMKKEDFDIYDEDENQSPRSFQKKTRHYFIAAVERLWDYGMSSSPHVLRNR AQSGSVPQFKKVVFQEFTDGSFTQPLYRGELNEHLGLLGPYIRAEVEDNIMVTFRNQASR PYSFYSSLISYEEDQRQGAEPRKNFVKPNETKTYFWKVQHHMAPTKDEFDCKAWAYFSDV DLEKDVHSGLIGPLLVCHTNTLNPAHGRQVTVQEFALFFTIFDETKSWYFTENMERNCRA PCNIQMEDPTFKENYRFHAINGYIMDTLPGLVMAQDQRIRWYLLSMGSNENIHSIHFSGH VFTVRKKEEYKMALYNLYPGVFETVEMLPSKAGIWRVECLIGEHLHAGMSTLFLVYSNKC QTPLGMASGHIRDFQITASGQYGQWAPKLARLHYSGSINAWSTKEPFSWIKVDLLAPMII HGIKTQGARQKFSSLYISQFIIMYSLDGKKWQTYRGNSTGTLMVFFGNVDSSGIKHNIFN PPIIARYIRLHPTHYSIRSTLRMELMGCDLNSCSMPLGMESKAISDAQITASSYFTNMFA TWSPSKARLHLQGRSNAWRPQVNNPKEWLQVDFQKTMKVTGVTTQGVKSLLTSMYVKEFL ISSSQDGHQWTLFFQNGKVKVFQGNQDSFTPVVNSLDPPLLTRYLRIHPQSWVHQIALRM EVLGCEAQDLY
Accession Number	P00451
PubMed ID	3935400, 6438526, 6438528, 1303178, 15772651, 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
CEX DB	HS_F8
CTD DB	2157
DIP DB	DIP-29774N
Ensembl DB	ENST00000360256
CATH	G3DSA:2.60.40.420
Genecard DB	GC0XM153717
GeneID DB	2157
GermOnline DB	ENSG00000185010
GO DB	0005615, 0005886, 0031093, 0005509, 0005507, 0016491, 0005515, 0006953, 0007596, 0007155, 0055114
HGNC DB	3546
HOGENOM DB	HBG127514
HPA DB	CAB018777
InterPro DB	IPR000421, IPR001117, IPR011706, IPR011707, IPR002355, IPR008972, IPR014707, IPR008979
H-InvDB	HIX0056228
IPI DB	IPI00017603
KEGG	hsa:2157
NCBI	M14113, AAA52485, X01179, CAA25619, K01740, AAA52484, M88648, AAA52420, M88628, M88629, M88630, M88631, M88632, M88633, M88634, M88635, M88636, M88638, M88639, M88640, M88641, M88642, M88643, M88644, M88645, M88646, M88647, AY769950, AAV85964, BX470111.4, CAI41660, AC109993.4, BX842559.3, BX842564.3, BX890586.2, CAI41666, CAI41672, CAI43241, U80228, AAB61261, NP_000123
OMA	HYFIAAV
OMIM	105200, 134820, 202400, 134830, 202400, 134850, 202400, 176930, 601367, 134390, 188055, 227400, 600880, 601367, 612309, 227500, 134500, 306700
Orphanet DB	448, 98878, 169808, 169805, 169802, 177926
OrthoDB	EOG9JQ6GW
PDB	1CFG_A, 1D7P_M, 1FAC_A, 1IQD_C, 2R7E_A, 2R7E_B, 3CDZ_A, 3CDZ_B
Pfam	PF00394, PF07731, PF07732, PF00754
PharmaGKB	PA134914298
PROSITE DB	PS01285, PS01286, PS50022, PS00079
SMART DB	SM00231
UCSC	uc004fmt.1
UniGene	Hs.654450