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Identification
NameAntihemophilic Factor (Recombinant)
Accession NumberDB00025  (BTD00029, BIOD00029)
TypeBiotech
GroupsApproved, Investigational
Description

Human recombinant antihemophilic factor (AHF) or Factor VIII, 2332 residues, glycosylated, produced by CHO cells

Protein structureDb00025
Related Articles
Protein chemical formulaC11794H18314N3220O3553S83
Protein average weight264725.5 Da
Sequences
>DB00025 sequence
ATRRYYLGAVELSWDYMQSDLGELPVDARFPPRVPKSFPFNTSVVYKKTLFVEFTDHLFN
IAKPRPPWMGLLGPTIQAEVYDTVVITLKNMASHPVSLHAVGVSYWKASEGAEYDDQTSQ
REKEDDKVFPGGSHTYVWQVLKENGPMASDPLCLTYSYLSHVDLVKDLNSGLIGALLVCR
EGSLAKEKTQTLHKFILLFAVFDEGKSWHSETKNSLMQDRDAASARAWPKMHTVNGYVNR
SLPGLIGCHRKSVYWHVIGMGTTPEVHSIFLEGHTFLVRNHRQASLEISPITFLTAQTLL
MDLGQFLLFCHISSHQHDGMEAYVKVDSCPEEPQLRMKNNEEAEDYDDDLTDSEMDVVRF
DDDNSPSFIQIRSVAKKHPKTWVHYIAAEEEDWDYAPLVLAPDDRSYKSQYLNNGPQRIG
RKYKKVRFMAYTDETFKTREAIQHESGILGPLLYGEVGDTLLIIFKNQASRPYNIYPHGI
TDVRPLYSRRLPKGVKHLKDFPILPGEIFKYKWTVTVEDGPTKSDPRCLTRYYSSFVNME
RDLASGLIGPLLICYKESVDQRGNQIMSDKRNVILFSVFDENRSWYLTENIQRFLPNPAG
VQLEDPEFQASNIMHSINGYVFDSLQLSVCLHEVAYWYILSIGAQTDFLSVFFSGYTFKH
KMVYEDTLTLFPFSGETVFMSMENPGLWILGCHNSDFRNRGMTALLKVSSCDKNTGDYYE
DSYEDISAYLLSKNNAIEPRSFSQNSRHPSTRQKQFNATTIPENDIEKTDPWFAHRTPMP
KIQNVSSSDLLMLLRQSPTPHGLSLSDLQEAKYETFSDDPSPGAIDSNNSLSEMTHFRPQ
LHHSGDMVFTPESGLQLRLNEKLGTTAATELKKLDFKVSSTSNNLISTIPSDNLAAGTDN
TSSLGPPSMPVHYDSQLDTTLFGKKSSPLTESGGPLSLSEENNDSKLLESGLMNSQESSW
GKNVSSTESGRLFKGKRAHGPALLTKDNALFKVSISLLKTNKTSNNSATNRKTHIDGPSL
LIENSPSVWQNILESDTEFKKVTPLIHDRMLMDKNATALRLNHMSNKTTSSKNMEMVQQK
KEGPIPPDAQNPDMSFFKMLFLPESARWIQRTHGKNSLNSGQGPSPKQLVSLGPEKSVEG
QNFLSEKNKVVVGKGEFTKDVGLKEMVFPSSRNLFLTNLDNLHENNTHNQEKKIQEEIEK
KETLIQENVVLPQIHTVTGTKNFMKNLFLLSTRQNVEGSYDGAYAPVLQDFRSLNDSTNR
TKKHTAHFSKKGEEENLEGLGNQTKQIVEKYACTTRISPNTSQQNFVTQRSKRALKQFRL
PLEETELEKRIIVDDTSTQWSKNMKHLTPSTLTQIDYNEKEKGAITQSPLSDCLTRSHSI
PQANRSPLPIAKVSSFPSIRPIYLTRVLFQDNSSHLPAASYRKKDSGVQESSHFLQGAKK
NNLSLAILTLEMTGDQREVGSLGTSATNSVTYKKVENTVLPKPDLPKTSGKVELLPKVHI
YQKDLFPTETSNGSPGHLDLVEGSLLQGTEGAIKWNEANRPGKVPFLRVATESSAKTPSK
LLDPLAWDNHYGTQIPKEEWKSQEKSPEKTAFKKKDTILSLNACESNHAIAAINEGQNKP
EIEVTWAKQGRTERLCSQNPPVLKRHQREITRTTLQSDQEEIDYDDTISVEMKKEDFDIY
DEDENQSPRSFQKKTRHYFIAAVERLWDYGMSSSPHVLRNRAQSGSVPQFKKVVFQEFTD
GSFTQPLYRGELNEHLGLLGPYIRAEVEDNIMVTFRNQASRPYSFYSSLISYEEDQRQGA
EPRKNFVKPNETKTYFWKVQHHMAPTKDEFDCKAWAYFSDVDLEKDVHSGLIGPLLVCHT
NTLNPAHGRQVTVQEFALFFTIFDETKSWYFTENMERNCRAPCNIQMEDPTFKENYRFHA
INGYIMDTLPGLVMAQDQRIRWYLLSMGSNENIHSIHFSGHVFTVRKKEEYKMALYNLYP
GVFETVEMLPSKAGIWRVECLIGEHLHAGMSTLFLVYSNKCQTPLGMASGHIRDFQITAS
GQYGQWAPKLARLHYSGSINAWSTKEPFSWIKVDLLAPMIIHGIKTQGARQKFSSLYISQ
FIIMYSLDGKKWQTYRGNSTGTLMVFFGNVDSSGIKHNIFNPPIIARYIRLHPTHYSIRS
TLRMELMGCDLNSCSMPLGMESKAISDAQITASSYFTNMFATWSPSKARLHLQGRSNAWR
PQVNNPKEWLQVDFQKTMKVTGVTTQGVKSLLTSMYVKEFLISSSQDGHQWTLFFQNGKV
KVFQGNQDSFTPVVNSLDPPLLTRYLRIHPQSWVHQIALRMEVLGCEAQDLY
Download FASTA Format
Synonyms
Antihemophilic factor (human)
Antihemophilic Factor (Recombinant), Plasma/Albumin-Free Method
Antihemophilic factor human
Antihemophilic Factor, Human Recombinant
Factor VIII (Recombinant)
Human Factor VIII (Recombinant)
rAHF
External Identifiers Not Available
Approved Prescription Products
NameDosageStrengthRouteLabellerMarketing StartMarketing End
Advatepowder for solution1500 unitintravenousBaxalta Canada Corporation2006-10-06Not applicableCanada
Advatepowder for solution1500 unitintravenousBaxalta Canada CorporationNot applicableNot applicableCanada
Advatepowder for solution250 unitintravenousBaxter Healthcare Corporation2006-10-062013-10-08Canada
Advatepowder for solution500 unitintravenousBaxalta Canada Corporation2012-03-05Not applicableCanada
Advatepowder for solution250 unitintravenousBaxalta Canada Corporation2012-01-16Not applicableCanada
Advatepowder for solution2000 unitintravenousBaxalta Canada Corporation2008-09-04Not applicableCanada
Advatepowder for solution1000 unitintravenousBaxalta Canada CorporationNot applicableNot applicableCanada
Advatepowder for solution3000 unitintravenousBaxalta Canada Corporation2010-01-05Not applicableCanada
Advatepowder for solution1000 unitintravenousBaxalta Canada Corporation2006-10-06Not applicableCanada
Advatepowder for solution500 unitintravenousBaxter Healthcare Corporation2006-10-062013-10-08Canada
AntiveninkitMerck Sharp & Dohme Corp.2014-12-01Not applicableUs
Factorate Inj 15unit/mlpowder for solution15 unitintravenousArmour Pharmaceutical Co.1984-12-311999-12-15Canada
Helixate FSpowder for solution3000 unitintravenousBayer HealthCare LLCNot applicableNot applicableCanada
Helixate FSpowder for solution2000 unitintravenousBayer HealthCare LLC2009-09-07Not applicableCanada
Helixate FSpowder for solution1000 unitintravenousBayer HealthCare LLC2008-04-24Not applicableCanada
Helixate FSpowder for solution500 unitintravenousBayer HealthCare LLC2008-04-24Not applicableCanada
Helixate FSpowder for solution250 unitintravenousBayer HealthCare LLC2008-04-24Not applicableCanada
Hemofil MkitBaxter Healthcare Corporation1988-02-23Not applicableUs
Hemofil MkitBaxter Healthcare Corporation1988-02-23Not applicableUs
Hemofil Mpowder for solution1700 unitintravenousBaxter Healthcare Corporation1988-12-312006-05-19Canada
Hemofil MkitBaxter Healthcare Corporation1988-02-23Not applicableUs
Hemofil MkitBaxter Healthcare Corporation1988-02-23Not applicableUs
Hyate C Inj Vialpowder for solution700 unitintravenousPorton Speywood Ltd.1986-12-311996-09-25Canada
Koate -dvikitKedrion Biopharma, Inc1999-05-20Not applicableUs
Koate -dvikitKedrion Biopharma, Inc1999-05-20Not applicableUs
Koate -dvikitKedrion Biopharma, Inc1999-05-20Not applicableUs
Koate-HP - Pws IV 1000iu/vialpowder for solution1000 unitintravenousBayer Corporation1997-01-232000-08-01Canada
Koate-HP Unjliquid1800 unitintravenousCutter Biological, Division Of Miles Lab Inc.1989-12-311998-09-25Canada
Kogenate - Pws IV 1000I.U./vialpowder for solution1000 unitintravenousBayer Corporation1996-07-092002-08-01Canada
Kogenate - Pws IV 250I.U./vialpowder for solution250 unitintravenousBayer Corporation1996-11-202002-08-01Canada
Kogenate - Pws IV 500I.U./vialpowder for solution500 unitintravenousBayer Corporation1996-11-202002-08-01Canada
Kogenate FSkitBayer Health Care Llc2000-06-26Not applicableUs
Kogenate FSkitBayer Health Care Llc2005-11-17Not applicableUs
Kogenate FSpowder for solution1000 unitintravenousBayer Inc2015-08-27Not applicableCanada
Kogenate FSpowder for solution1000 unitintravenousBayer Inc2000-09-292009-08-06Canada
Kogenate FSpowder for solution500 unitintravenousBayer Inc2015-08-13Not applicableCanada
Kogenate FSkitBayer Health Care Llc2005-11-17Not applicableUs
Kogenate FSkitBayer Health Care Llc2009-07-31Not applicableUs
Kogenate FSpowder for solution500 unitintravenousBayer Inc2000-09-292009-08-06Canada
Kogenate FSpowder for solution250 unitintravenousBayer Inc2015-10-14Not applicableCanada
Kogenate FSkitBayer Health Care Llc2007-06-07Not applicableUs
Kogenate FSpowder for solution250 unitintravenousBayer Inc2004-03-312009-08-06Canada
Kogenate FSkitBayer Health Care Llc2009-07-31Not applicableUs
Kogenate FSpowder for solution1000 unitintravenousBayer Inc2005-09-262015-08-27Canada
Kogenate FSkitBayer Health Care Llc2000-06-26Not applicableUs
Kogenate FSkitBayer Health Care Llc2008-07-04Not applicableUs
Kogenate FSpowder for solution500 unitintravenousBayer Inc2005-09-262015-08-13Canada
Kogenate FSkitBayer Health Care Llc2000-06-26Not applicableUs
Kogenate FSkitBayer Health Care Llc2005-11-17Not applicableUs
Kogenate FSpowder for solution250 unitintravenousBayer Inc2006-08-042015-10-14Canada
Kogenate FS -(with Bio-set)powder for solution3000 unitintravenousBayer Inc2010-05-112015-07-07Canada
Kogenate FS -(with Bio-set)powder for solution2000 unitintravenousBayer Inc2008-08-06Not applicableCanada
Kogenate FS -(with Vial Adapter)powder for solution2000 unitintravenousBayer IncNot applicableNot applicableCanada
Kogenate FS -(with Vial Adapter)powder for solution3000 unitintravenousBayer Inc2015-07-07Not applicableCanada
Kogenate Pws 1000iu/vialpowder for solution1000 unitintravenousMiles Inc. Pharmaceutical Division1993-12-311998-09-25Canada
Kogenate Pws 250iu/vialpowder for solution250 unitintravenousMiles Inc. Pharmaceutical Division1993-12-311998-09-25Canada
Kogenate Pws 500iu/vialpowder for solution500 unitintravenousMiles Inc. Pharmaceutical Division1993-12-311998-09-25Canada
KovaltrykitBayer Health Care Llc2016-03-17Not applicableUs
Kovaltrykit; powder for solution3000 unitintravenousBayer IncNot applicableNot applicableCanada
Kovaltrykit; powder for solution500 unitintravenousBayer IncNot applicableNot applicableCanada
KovaltrykitBayer Health Care Llc2016-03-17Not applicableUs
Kovaltrykit; powder for solution2000 unitintravenousBayer IncNot applicableNot applicableCanada
KovaltrykitBayer Health Care Llc2016-03-17Not applicableUs
Kovaltrykit; powder for solution1000 unitintravenousBayer IncNot applicableNot applicableCanada
KovaltrykitBayer Health Care Llc2016-03-17Not applicableUs
Kovaltrykit; powder for solution500 unitintravenousBayer IncNot applicableNot applicableCanada
Kovaltrykit; powder for solution3000 unitintravenousBayer IncNot applicableNot applicableCanada
Kovaltrykit; powder for solution250 unitintravenousBayer IncNot applicableNot applicableCanada
Kovaltrykit; powder for solution2000 unitintravenousBayer IncNot applicableNot applicableCanada
KovaltrykitBayer Health Care Llc2016-03-17Not applicableUs
Kovaltrykit; powder for solution250 unitintravenousBayer IncNot applicableNot applicableCanada
Kovaltrykit; powder for solution1000 unitintravenousBayer IncNot applicableNot applicableCanada
Kryobulin Vh 1000 Unit/vialpowder for solution1000 unitintravenousOsterreichisches Institut Fur Haemoderivate Ges M.B.H.1988-12-311998-08-13Canada
Kryobulin Vh 500 Unit/vialpowder for solution500 unitintravenousOsterreichisches Institut Fur Haemoderivate Ges M.B.H.1987-12-311998-08-13Canada
Monoclate-PkitCSL Behring LLC2004-03-04Not applicableUs
Monoclate-PkitCSL Behring LLC1990-05-30Not applicableUs
Monoclate-PkitCSL Behring LLC1990-05-30Not applicableUs
Monoclate-PkitCSL Behring LLC1990-05-30Not applicableUs
NovoeightkitNovo Nordisk2015-04-01Not applicableUs
NovoeightkitNovo Nordisk2015-04-01Not applicableUs
NovoeightkitNovo Nordisk2015-04-01Not applicableUs
NovoeightkitNovo Nordisk2015-04-01Not applicableUs
NovoeightkitNovo Nordisk2015-04-01Not applicableUs
NovoeightkitNovo Nordisk2015-04-01Not applicableUs
NuwiqkitintravenousOctapharma Usa Inc2016-01-01Not applicableUs
NuwiqkitintravenousOctapharma Usa Inc2016-01-01Not applicableUs
NuwiqkitintravenousOctapharma Usa Inc2016-01-01Not applicableUs
NuwiqkitintravenousOctapharma Usa Inc2016-01-01Not applicableUs
Profilate Heat Treated-wet Method Injliquid1000 unitintravenousAlpha Therapeutic Corporation1988-12-311997-11-12Canada
RecombinatekitBaxter Healthcare Corporation2010-02-08Not applicableUs
RecombinatekitBaxter Healthcare Corporation2010-03-10Not applicableUs
RecombinatekitBaxter Healthcare Corporation2010-02-08Not applicableUs
RecombinatekitBaxter Healthcare Corporation2010-03-10Not applicableUs
RecombinatekitBaxter Healthcare Corporation1992-12-10Not applicableUs
RecombinatekitBaxter Healthcare Corporation1992-12-10Not applicableUs
RecombinatekitBaxter Healthcare Corporation1992-12-10Not applicableUs
RecombinatekitBaxter Healthcare Corporation2010-02-08Not applicableUs
Recombinate Pws Inj 1000I.U./vialpowder for solution1000 unitintravenousBaxter Healthcare Corporation1992-12-312009-02-12Canada
Recombinate Pws Inj 250I.U./vialpowder for solution250 unitintravenousBaxter Healthcare Corporation1992-12-312009-02-12Canada
Recombinate Pws Inj 500I.U./vialpowder for solution500 unitintravenousBaxter Healthcare Corporation1992-12-312009-02-12Canada
XynthakitWyeth Bio Pharma Division Of Wyeth Pharmaceuticals Inc., A Subsidiary Of Pfizer Inc.2008-08-01Not applicableUs
XynthakitWyeth Bio Pharma Division Of Wyeth Pharmaceuticals Inc., A Subsidiary Of Pfizer Inc.2008-08-01Not applicableUs
XynthakitWyeth Bio Pharma Division Of Wyeth Pharmaceuticals Inc., A Subsidiary Of Pfizer Inc.2011-08-01Not applicableUs
XynthakitWyeth Bio Pharma Division Of Wyeth Pharmaceuticals Inc., A Subsidiary Of Pfizer Inc.2011-08-01Not applicableUs
XynthakitWyeth Bio Pharma Division Of Wyeth Pharmaceuticals Inc., A Subsidiary Of Pfizer Inc.2011-12-01Not applicableUs
XynthakitWyeth Bio Pharma Division Of Wyeth Pharmaceuticals Inc., A Subsidiary Of Pfizer Inc.2011-12-01Not applicableUs
XynthakitWyeth Bio Pharma Division Of Wyeth Pharmaceuticals Inc., A Subsidiary Of Pfizer Inc.2008-08-01Not applicableUs
XynthakitWyeth Bio Pharma Division Of Wyeth Pharmaceuticals Inc., A Subsidiary Of Pfizer Inc.2010-08-13Not applicableUs
XynthakitWyeth Bio Pharma Division Of Wyeth Pharmaceuticals Inc., A Subsidiary Of Pfizer Inc.2008-08-01Not applicableUs
Approved Generic Prescription ProductsNot Available
Approved Over the Counter ProductsNot Available
Unapproved/Other Products Not Available
International Brands
NameCompany
BioclateNot Available
HelixateNot Available
Hyate:CNot Available
Koate-HPNot Available
KogenateNot Available
Monarc-MNot Available
ReFactoNot Available
Brand mixtures
NameLabellerIngredients
AdvateBaxter Healthcare Corporation
Helixate FSCSL Behring LLC
Monoclate-P - 200-350iu/vialAventis Behring Llc
Monoclate-P - 400-700iu/vialAventis Behring Llc
Monoclate-P - 800-1500iu/vialAventis Behring Llc
SaltsNot Available
Categories
UNII839MOZ74GK
CAS numberNot Available
Taxonomy
DescriptionNot Available
KingdomOrganic Compounds
Super ClassOrganic Acids
ClassCarboxylic Acids and Derivatives
Sub ClassAmino Acids, Peptides, and Analogues
Direct ParentPeptides
Alternative ParentsNot Available
SubstituentsNot Available
Molecular FrameworkNot Available
External DescriptorsNot Available
Pharmacology
IndicationFor the treatment of hemophilia A, von Willebrand disease and Factor XIII deficiency.
PharmacodynamicsAntihemophilic Factor binds factor IXa along with calcium and phospholipid, This complex converts factor X to factor Xa to facilitate clotting cascade.
Mechanism of actionAntihemophilic factor (AHF) is a protein found in normal plasma which is necessary for clot formation. The administration of AHF provides an increase in plasma levels of AHF and can temporarily correct the coagulation defect of patients with hemophilia A (classical hemophilia).
Related Articles
AbsorptionNot Available
Volume of distributionNot Available
Protein bindingNot Available
MetabolismNot Available
Route of eliminationNot Available
Half life8.4-19.3 hrs
Clearance
  • 4.1 mL/h•kg [Previously treated pediatric patients]
ToxicityNot Available
Affected organisms
  • Humans and other mammals
PathwaysNot Available
SNP Mediated EffectsNot Available
SNP Mediated Adverse Drug ReactionsNot Available
Pharmacoeconomics
ManufacturersNot Available
Packagers
Dosage forms
FormRouteStrength
Powder for solutionintravenous1500 unit
Powder for solutionintravenous3000 unit
Powder for solutionintravenous15 unit
Kit
Powder for solutionintravenous1000 unit
Powder for solutionintravenous250 unit
Powder for solutionintravenous500 unit
Powder for solutionintravenous1700 unit
Powder for solutionintravenous700 unit
Liquidintravenous1800 unit
Kit
Powder for solutionintravenous2000 unit
Kit; powder for solutionintravenous1000 unit
Kit; powder for solutionintravenous2000 unit
Kit; powder for solutionintravenous250 unit
Kit; powder for solutionintravenous3000 unit
Kit; powder for solutionintravenous500 unit
Solution; kitintravenous
Kitintravenous
Liquidintravenous1000 unit
Kit
Prices
Unit descriptionCostUnit
Advate 1201-1800 unit vial1.68USD vial
Advate 1801-2400 unit vial1.68USD vial
Advate 200-400 unit vial1.68USD vial
Advate 2400-3600 unit vial1.68USD vial
Advate 401-800 unit vial1.68USD vial
Advate 801-1200 unit vial1.68USD vial
Kogenate fs 1000 unit vial1.68USD vial
Kogenate fs 250 unit vial1.68USD vial
Kogenate fs 3000 unit vial1.68USD vial
Kogenate fs 500 unit vial1.68USD vial
Xyntha 1000 unit kit1.66USD kit
Xyntha 2000 unit kit1.66USD kit
Xyntha 250 unit kit1.66USD kit
Xyntha 500 unit kit1.66USD kit
Helixate fs 1000 unit vial1.56USD vial
Helixate fs 250 unit vial1.56USD vial
Helixate fs 3000 unit vial1.56USD vial
Helixate fs 500 unit vial1.56USD vial
Wilate 450-450 unit kit1.38USD kit
Wilate 900-900 unit kit1.38USD kit
Hemofil m 1701-2000 unit vial1.34USD vial
Hemofil m 220-400 unit vial1.34USD vial
Hemofil m 401-800 unit vial1.34USD vial
Hemofil m 801-1700 unit vial1.34USD vial
Koate-dvi 1000 unit kit1.31USD kit
Koate-dvi 250 unit kit1.31USD kit
Koate-dvi 500 unit kit1.31USD kit
Refacto 1000 unit vial1.31USD vial
Refacto 2000 unit vial1.31USD vial
Refacto 250 unit vial1.31USD vial
Refacto 500 unit vial1.31USD vial
Alphanate 1000-1500 unit vial1.2USD vial
Alphanate 250-500 unit vial1.2USD vial
Humate-p 1000 unit kit1.2USD kit
Humate-p 1200 unit kit1.2USD kit
Humate-p 2000 unit kit1.2USD kit
Humate-p 2400 unit kit1.2USD kit
Humate-p 500 unit kit1.2USD kit
Humate-p 600 unit kit1.2USD kit
Monoclate-p 1000 unit kit1.01USD kit
Monoclate-p 1500 unit kit1.01USD kit
Monoclate-p 250 unit kit1.01USD kit
Monoclate-p 500ahfu kit1.01USD kit
DrugBank does not sell nor buy drugs. Pricing information is supplied for informational purposes only.
Patents
Patent NumberPediatric ExtensionApprovedExpires (estimated)
CA1339477 No1997-09-232014-09-23Canada
CA2124690 No2007-09-112013-10-01Canada
Properties
StateSolid
Experimental Properties
PropertyValueSource
hydrophobicity-0.533Not Available
isoelectric point6.97Not Available
References
Synthesis Reference

James W. Bloom, “Warm ethanol method for preparation of low fibrinogen antihemophilic factor.” U.S. Patent US4478825, issued June, 1955.

US4478825
General References
  1. Titheradge MA, Coore HG: Initial rates of pyruvate transport in mitochondria determined by an "inhibitor-stop" technique. Biochem J. 1975 Sep;150(3):553-6. [PubMed:2157 ]
External Links
ATC CodesNot Available
AHFS Codes
  • 20:28.16
PDB Entries
FDA labelNot Available
MSDSNot Available
Interactions
Drug InteractionsNo interactions found.
Food InteractionsNot Available

Targets

Kind
Protein
Organism
Human
Pharmacological action
yes
Actions
activator
General Function:
Serine-type endopeptidase activity
Specific Function:
Factor Xa is a vitamin K-dependent glycoprotein that converts prothrombin to thrombin in the presence of factor Va, calcium and phospholipid during blood clotting.
Gene Name:
F10
Uniprot ID:
P00742
Molecular Weight:
54731.255 Da
References
  1. BLATRIX C, SOULIER JP: [Preparation of a fraction rich in prothrombin, proconvertin, Stuart factor and antihemophilic factor B (P.P.B. fraction)]. Pathol Biol (Paris). 1959 Dec;7:2477-86. [PubMed:13801371 ]
  2. LUNDBLAD RL, DAVIE EW: THE ACTIVATION OF STUART FACTOR (FACTOR X) BY ACTIVATED ANTIHEMOPHILIC FACTOR (ACTIVATED FACTOR 8). Biochemistry. 1965 Jan;4:113-20. [PubMed:14285227 ]
  3. Radnoff OD, Saito H: Inhibition of Hageman factor, plasma thromboplastin antecedent, thrombin and other clotting factors by phenylglyoxal hydrate (38500). Proc Soc Exp Biol Med. 1975 Jan;148(1):177-82. [PubMed:236567 ]
  4. Orthner CL: Characterization of proteases in AHF concentrates: effect on factor VIII:von Willebrand protein as assessed by high-pressure gel permeation chromatography. J Lab Clin Med. 1984 Nov;104(5):816-28. [PubMed:6436416 ]
  5. Freedman J, Mody M, Lazarus AH, Dewar L, Song S, Blanchette VS, Garvey MB, Ofosu FA: Platelet activation and hypercoagulability following treatment with porcine factor VIII (HYATE:C). Am J Hematol. 2002 Mar;69(3):192-9. [PubMed:11891806 ]
Kind
Protein
Organism
Human
Pharmacological action
yes
Actions
cofactor
General Function:
Serine-type endopeptidase activity
Specific 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.
Gene Name:
F9
Uniprot ID:
P00740
Molecular Weight:
51778.11 Da
References
  1. Hule V: [Factor IX inhibitor (antihemophilic factor B, PTC) in a woman]. Vnitr Lek. 1975 Mar;21(3):274-7. [PubMed:1119107 ]
  2. Yoshitake S, Schach BG, Foster DC, Davie EW, Kurachi K: Nucleotide sequence of the gene for human factor IX (antihemophilic factor B). Biochemistry. 1985 Jul 2;24(14):3736-50. [PubMed:2994716 ]
  3. LUNDBLAD RL, DAVIE EW: THE ACTIVATION OF ANTIHEMOPHILIC FACTOR (FACTOR 8) BY ACTIVATED CHRISTMAS FACTOR (ACTIVATED FACTOR9 9). Biochemistry. 1964 Nov;3:1720-5. [PubMed:14240634 ]
  4. Hoofnagle JH, Gerety RJ, Thiel J, Barker LF: The prevalence of hepatitis B surface antigen in commercially prepared plasma products. J Lab Clin Med. 1976 Jul;88(1):102-13. [PubMed:932529 ]
  5. Prince AM, Horowitz B, Brotman B, Huima T, Richardson L, van den Ende MC: Inactivation of hepatitis B and Hutchinson strain non-A, non-B hepatitis viruses by exposure to Tween 80 and ether. Vox Sang. 1984;46(1):36-43. [PubMed:6422634 ]
Kind
Protein
Organism
Human
Pharmacological action
yes
Actions
binder
General Function:
Protein n-terminus binding
Specific Function:
Important in the maintenance of hemostasis, it promotes adhesion of platelets to the sites of vascular injury by forming a molecular bridge between sub-endothelial collagen matrix and platelet-surface receptor complex GPIb-IX-V. Also acts as a chaperone for coagulation factor VIII, delivering it to the site of injury, stabilizing its heterodimeric structure and protecting it from premature clea...
Gene Name:
VWF
Uniprot ID:
P04275
Molecular Weight:
309261.83 Da
References
  1. Shord SS, Lindley CM: Coagulation products and their uses. Am J Health Syst Pharm. 2000 Aug 1;57(15):1403-17; quiz 1418-20. [PubMed:10938981 ]
  2. Lillicrap D, Poon MC, Walker I, Xie F, Schwartz BA: Efficacy and safety of the factor VIII/von Willebrand factor concentrate, haemate-P/humate-P: ristocetin cofactor unit dosing in patients with von Willebrand disease. Thromb Haemost. 2002 Feb;87(2):224-30. [PubMed:11858481 ]
  3. Gill JC, Ewenstein BM, Thompson AR, Mueller-Velten G, Schwartz BA: Successful treatment of urgent bleeding in von Willebrand disease with factor VIII/VWF concentrate (Humate-P): use of the ristocetin cofactor assay (VWF:RCo) to measure potency and to guide therapy. Haemophilia. 2003 Nov;9(6):688-95. [PubMed:14750934 ]
  4. Smith KJ, Lusher JM, Cohen AR, Salzman P: Initial clinical experience with a new pasteurized monoclonal antibody purified factor VIIIC. Semin Hematol. 1990 Apr;27(2 Suppl 2):25-9. [PubMed:2128855 ]
  5. Altieri DC, Capitanio AM, Mannucci PM: von Willebrand factor contaminating porcine factor VIII concentrate (Hyate:C) causes platelet aggregation. Br J Haematol. 1986 Aug;63(4):703-11. [PubMed:2942172 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
antagonist
General Function:
Phytanoyl-coa dioxygenase activity
Specific Function:
Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
Gene Name:
PHYH
Uniprot ID:
O14832
Molecular Weight:
38538.065 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
  3. Chen C, Wang Q, Fang X, Xu Q, Chi C, Gu J: Roles of phytanoyl-CoA alpha-hydroxylase in mediating the expression of human coagulation factor VIII. J Biol Chem. 2001 Dec 7;276(49):46340-6. [PubMed:11574539 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
binder
General Function:
Carbohydrate binding
Specific Function:
Mediates the endocytosis of plasma glycoproteins to which the terminal sialic acid residue on their complex carbohydrate moieties has been removed. The receptor recognizes terminal galactose and N-acetylgalactosamine units. After ligand binding to the receptor, the resulting complex is internalized and transported to a sorting organelle, where receptor and ligand are disassociated. The receptor...
Gene Name:
ASGR2
Uniprot ID:
P07307
Molecular Weight:
35092.04 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
  3. Bovenschen N, Rijken DC, Havekes LM, van Vlijmen BJ, Mertens K: The B domain of coagulation factor VIII interacts with the asialoglycoprotein receptor. J Thromb Haemost. 2005 Jun;3(6):1257-65. [PubMed:15946216 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
chaperone
General Function:
Unfolded protein binding
Specific Function:
Probably plays a role in facilitating the assembly of multimeric protein complexes inside the endoplasmic reticulum. Involved in the correct folding of proteins and degradation of misfolded proteins via its interaction with DNAJC10, probably to facilitate the release of DNAJC10 from its substrate.
Gene Name:
HSPA5
Uniprot ID:
P11021
Molecular Weight:
72332.425 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
  3. Kaufman RJ, Pipe SW, Tagliavacca L, Swaroop M, Moussalli M: Biosynthesis, assembly and secretion of coagulation factor VIII. Blood Coagul Fibrinolysis. 1997 Dec;8 Suppl 2:S3-14. [PubMed:9607108 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
chaperone
General Function:
Zinc ion binding
Specific Function:
Calcium-binding chaperone that promotes folding, oligomeric assembly and quality control in the endoplasmic reticulum (ER) via the calreticulin/calnexin cycle. This lectin interacts transiently with almost all of the monoglucosylated glycoproteins that are synthesized in the ER. Interacts with the DNA-binding domain of NR3C1 and mediates its nuclear export. Involved in maternal gene expression ...
Gene Name:
CALR
Uniprot ID:
P27797
Molecular Weight:
48141.2 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
  3. Pipe SW, Morris JA, Shah J, Kaufman RJ: Differential interaction of coagulation factor VIII and factor V with protein chaperones calnexin and calreticulin. J Biol Chem. 1998 Apr 3;273(14):8537-44. [PubMed:9525969 ]
  4. Srour MA, Grupp J, Aburubaiha Z, Albert T, Brondke H, Oldenburg J, Schwaab R: Modified expression of coagulation factor VIII by addition of a glycosylation site at the N terminus of the protein. Ann Hematol. 2008 Feb;87(2):107-12. Epub 2007 Sep 26. [PubMed:17899080 ]
  5. Kaufman RJ, Pipe SW, Tagliavacca L, Swaroop M, Moussalli M: Biosynthesis, assembly and secretion of coagulation factor VIII. Blood Coagul Fibrinolysis. 1997 Dec;8 Suppl 2:S3-14. [PubMed:9607108 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
chaperone
General Function:
Poly(a) rna binding
Specific Function:
Calcium-binding protein that interacts with newly synthesized glycoproteins in the endoplasmic reticulum. It may act in assisting protein assembly and/or in the retention within the ER of unassembled protein subunits. It seems to play a major role in the quality control apparatus of the ER by the retention of incorrectly folded proteins. Associated with partial T-cell antigen receptor complexes...
Gene Name:
CANX
Uniprot ID:
P27824
Molecular Weight:
67567.695 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
  3. Pipe SW, Morris JA, Shah J, Kaufman RJ: Differential interaction of coagulation factor VIII and factor V with protein chaperones calnexin and calreticulin. J Biol Chem. 1998 Apr 3;273(14):8537-44. [PubMed:9525969 ]
  4. Becker S, Simpson JC, Pepperkok R, Heinz S, Herder C, Grez M, Seifried E, Tonn T: Confocal microscopy analysis of native, full length and B-domain deleted coagulation factor VIII trafficking in mammalian cells. Thromb Haemost. 2004 Jul;92(1):23-35. [PubMed:15213841 ]
  5. Srour MA, Grupp J, Aburubaiha Z, Albert T, Brondke H, Oldenburg J, Schwaab R: Modified expression of coagulation factor VIII by addition of a glycosylation site at the N terminus of the protein. Ann Hematol. 2008 Feb;87(2):107-12. Epub 2007 Sep 26. [PubMed:17899080 ]
  6. Kaufman RJ, Pipe SW, Tagliavacca L, Swaroop M, Moussalli M: Biosynthesis, assembly and secretion of coagulation factor VIII. Blood Coagul Fibrinolysis. 1997 Dec;8 Suppl 2:S3-14. [PubMed:9607108 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
chaperone
General Function:
Unfolded protein binding
Specific Function:
Mannose-specific lectin. May recognize sugar residues of glycoproteins, glycolipids, or glycosylphosphatidyl inositol anchors and may be involved in the sorting or recycling of proteins, lipids, or both. The LMAN1-MCFD2 complex forms a specific cargo receptor for the ER-to-Golgi transport of selected proteins.
Gene Name:
LMAN1
Uniprot ID:
P49257
Molecular Weight:
57548.665 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
  3. Cunningham MA, Pipe SW, Zhang B, Hauri HP, Ginsburg D, Kaufman RJ: LMAN1 is a molecular chaperone for the secretion of coagulation factor VIII. J Thromb Haemost. 2003 Nov;1(11):2360-7. [PubMed:14629470 ]
  4. Miao HZ, Sirachainan N, Palmer L, Kucab P, Cunningham MA, Kaufman RJ, Pipe SW: Bioengineering of coagulation factor VIII for improved secretion. Blood. 2004 May 1;103(9):3412-9. Epub 2004 Jan 15. [PubMed:14726380 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
modulator
General Function:
Receptor activity
Specific Function:
Endocytic receptor involved in endocytosis and in phagocytosis of apoptotic cells. Required for early embryonic development. Involved in cellular lipid homeostasis. Involved in the plasma clearance of chylomicron remnants and activated LRPAP1 (alpha 2-macroglobulin), as well as the local metabolism of complexes between plasminogen activators and their endogenous inhibitors. May modulate cellula...
Gene Name:
LRP1
Uniprot ID:
Q07954
Molecular Weight:
504601.695 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
  3. Franchini M, Montagnana M: Low-density lipoprotein receptor-related protein 1: new functions for an old molecule. Clin Chem Lab Med. 2011 Jun;49(6):967-70. doi: 10.1515/CCLM.2011.154. Epub 2011 Mar 11. [PubMed:21391865 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
modulator
General Function:
Calcium ion binding
Specific Function:
The MCFD2-LMAN1 complex forms a specific cargo receptor for the ER-to-Golgi transport of selected proteins. Plays a role in the secretion of coagulation factors.
Gene Name:
MCFD2
Uniprot ID:
Q8NI22
Molecular Weight:
16390.175 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
  3. Zhang B, Kaufman RJ, Ginsburg D: LMAN1 and MCFD2 form a cargo receptor complex and interact with coagulation factor VIII in the early secretory pathway. J Biol Chem. 2005 Jul 8;280(27):25881-6. Epub 2005 May 10. [PubMed:15886209 ]

Enzymes

Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
activator
General Function:
Thrombospondin receptor activity
Specific Function:
Thrombin, which cleaves bonds after Arg and Lys, converts fibrinogen to fibrin and activates factors V, VII, VIII, XIII, and, in complex with thrombomodulin, protein C. Functions in blood homeostasis, inflammation and wound healing.
Gene Name:
F2
Uniprot ID:
P00734
Molecular Weight:
70036.295 Da
References
  1. Alberio L, Safa O, Clemetson KJ, Esmon CT, Dale GL: Surface expression and functional characterization of alpha-granule factor V in human platelets: effects of ionophore A23187, thrombin, collagen, and convulxin. Blood. 2000 Mar 1;95(5):1694-702. [PubMed:10688826 ]
  2. Ratnoff OD, Lewis JH: Heckathorn's disease: variable functional dificiency of antihemophilic factor (factor VIII). Blood. 1975 Aug;46(2):161-73. [PubMed:1139038 ]
  3. Anderson DM, Shelley S, Crick N, Buraglio M: No effect of the novel antidiabetic agent nateglinide on the pharmacokinetics and anticoagulant properties of warfarin in healthy volunteers. J Clin Pharmacol. 2002 Dec;42(12):1358-65. [PubMed:12463731 ]
  4. Piet MP, Chin S, Prince AM, Brotman B, Cundell AM, Horowitz B: The use of tri(n-butyl)phosphate detergent mixtures to inactivate hepatitis viruses and human immunodeficiency virus in plasma and plasma's subsequent fractionation. Transfusion. 1990 Sep;30(7):591-8. [PubMed:2402772 ]
  5. Lazarchick J, Ashby MA, Lazarchick JJ, Sens DA: Mechanism of factor VIII inactivation by human antibodies. IV. Antibody binding prevents factor VIII proteolysis by thrombin. Ann Clin Lab Sci. 1986 Nov-Dec;16(6):497-501. [PubMed:3099625 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
inactivator
General Function:
Serine-type endopeptidase activity
Specific Function:
Protein C is a vitamin K-dependent serine protease that regulates blood coagulation by inactivating factors Va and VIIIa in the presence of calcium ions and phospholipids (PubMed:25618265). Exerts a protective effect on the endothelial cell barrier function (PubMed:25651845).
Gene Name:
PROC
Uniprot ID:
P04070
Molecular Weight:
52070.82 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
  3. Bereczky Z, Kovacs KB, Muszbek L: Protein C and protein S deficiencies: similarities and differences between two brothers playing in the same game. Clin Chem Lab Med. 2010 Dec;48 Suppl 1:S53-66. doi: 10.1515/CCLM.2010.369. Epub 2010 Nov 5. [PubMed:21054189 ]
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Drug created on June 13, 2005 07:24 / Updated on April 25, 2016 11:21