Antihemophilic factor, human recombinant

Identification

Summary

Antihemophilic factor, human recombinant is a form of recombinant coagulation Factor VIII used to treat hemophilia A, von Willebrand disease, and Factor XIII deficiency.

Brand Names
Advate, Adynovate, Helixate, Kogenate, Kovaltry, Novoeight, Recombinate
Generic Name
Antihemophilic factor, human recombinant
DrugBank Accession Number
DB00025
Background

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

Type
Biotech
Groups
Approved, Investigational
Biologic Classification
Protein Based Therapies
Blood factors
Protein Structure
Protein Chemical Formula
C11794H18314N3220O3553S83
Protein Average Weight
264725.5 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 (recombinant)
  • Antihemophilic factor recombinant
  • Antihemophilic factor, human recombinant
  • Antihemophilic factor, recombinant
  • Coagulation factor VIII, recombinant
  • Factor VIII (rDNA)
  • Factor VIII (Recombinant)
  • Factor VIII recombin
  • Factor VIII, recombinant
  • Human Factor VIII (Recombinant)
  • Human factor VIII recombinant
  • Octocog alfa
  • rAHF
  • Recombinant antihemophilic factor VIII
External IDs
  • BAY 14-2222
  • BAY 81-8973
  • BAY W 6240
  • BAY-14-2222
  • BAY-81-8973
  • BAY-W-6240
  • BAY81-8973

Pharmacology

Indication

The human recombinant antihemophilic factor is indicated for use in adults and children with hemophilia A for the control and prevention of bleeding episodes, perioperative management, and routine prophylaxis to prevent or reduce the frequency of bleeding episodes.2,3

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Associated Conditions
Indication TypeIndicationCombined Product DetailsApproval LevelAge GroupPatient CharacteristicsDose Form
Management ofBleeding••••••••••••••••••••••• •••••• ••••••• ••••••••••••••••••
Prophylaxis ofBleeding••••••••••••••••••••••• •••••• ••••••• ••••••••••••••••••
Prevention ofBleeding••••••••••••••••••••••• •••••• ••••••• ••••••••••••••••••
Prevention ofJoint damage••••••••••••••••••••••••••••••• ••
Management ofPerioperative bleeding••••••••••••••••••••••• •••••• ••••••• ••••••••••••••••••
Contraindications & Blackbox Warnings
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Pharmacodynamics

Antihemophilic Factor binds factor IXa along with calcium and phospholipid, This complex converts factor X to factor Xa to facilitate clotting cascade.

Mechanism of action

Antihemophilic 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).

TargetActionsOrganism
ACoagulation factor X
activator
Humans
ACoagulation factor IX
cofactor
Humans
Avon Willebrand factor
binder
Humans
UPhytanoyl-CoA dioxygenase, peroxisomal
antagonist
Humans
UAsialoglycoprotein receptor 2
binder
Humans
UEndoplasmic reticulum chaperone BiP
chaperone
Humans
UCalreticulin
chaperone
Humans
UCalnexin
chaperone
Humans
UProtein ERGIC-53
chaperone
Humans
UProlow-density lipoprotein receptor-related protein 1
modulator
Humans
UMultiple coagulation factor deficiency protein 2
modulator
Humans
Absorption

Not Available

Volume of distribution

Not Available

Protein binding

Not Available

Metabolism
Not Available
Route of elimination

Not Available

Half-life

8.4-19.3 hrs

Clearance
  • 4.1 mL/h•kg [Previously treated pediatric patients]
Adverse Effects
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Toxicity

Not Available

Pathways
Not Available
Pharmacogenomic Effects/ADRs
Not Available

Interactions

Drug Interactions
This information should not be interpreted without the help of a healthcare provider. If you believe you are experiencing an interaction, contact a healthcare provider immediately. The absence of an interaction does not necessarily mean no interactions exist.
DrugInteraction
AbciximabThe therapeutic efficacy of Antihemophilic factor, human recombinant can be decreased when used in combination with Abciximab.
AcenocoumarolThe therapeutic efficacy of Antihemophilic factor, human recombinant can be decreased when used in combination with Acenocoumarol.
Alpha-1-proteinase inhibitorAlpha-1-proteinase inhibitor may increase the thrombogenic activities of Antihemophilic factor, human recombinant.
AlteplaseThe therapeutic efficacy of Antihemophilic factor, human recombinant can be decreased when used in combination with Alteplase.
Aminocaproic acidThe risk or severity of adverse effects can be increased when Aminocaproic acid is combined with Antihemophilic factor, human recombinant.
Food Interactions
No interactions found.

Products

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International/Other Brands
Bioclate / Helixate / Hyate:C / Koate-HP / Kogenate / Monarc-M / ReFacto
Brand Name Prescription Products
NameDosageStrengthRouteLabellerMarketing StartMarketing EndRegionImage
AdvatePowder, for solution1000 unit / vialIntravenousTakeda Italia S.P.A.Not applicableNot applicableCanada flag
AdvateInjection, powder, for solution500 IUIntravenousTakeda Manufacturing Austria Ag2016-09-07Not applicableEU flag
AdvateInjection, powder, for solution1000 IUIntravenousTakeda Manufacturing Austria Ag2016-09-07Not applicableEU flag
AdvateInjection, powder, lyophilized, for solution; Kit750 [iU]/1mLIntravenousTakeda Pharma A/S2011-12-15Not applicableUS flag
AdvateKit250 [iU]/1mLIntravenousBaxalta US Inc.2012-07-122016-08-26US flag
Mixture Products
NameIngredientsDosageRouteLabellerMarketing StartMarketing EndRegionImage
ALPHANATEAntihemophilic factor, human recombinant (500 IU) + Von Willebrand factor human (600 IU)Injection, powder, lyophilized, for solutionParenteralGRIFOLS USA, LLC2014-07-08Not applicableItaly flag
FANHDIAntihemophilic factor, human recombinant (500 IU/10mL) + Von Willebrand factor human (600 IU/10mL)Injection, powder, lyophilized, for solutionIntravenousInstituto Grifols, S.A.2014-07-08Not applicableItaly flag
FANHDIAntihemophilic factor, human recombinant (1000 IU/10mL) + Von Willebrand factor human (1200 IU/10mL)Injection, powder, lyophilized, for solutionIntravenousInstituto Grifols, S.A.2014-07-08Not applicableItaly flag
FANHDIAntihemophilic factor, human recombinant (250 IU/10mL) + Von Willebrand factor human (300 IU/10mL)Injection, powder, lyophilized, for solutionIntravenousInstituto Grifols, S.A.2014-07-08Not applicableItaly flag
FANHDIAntihemophilic factor, human recombinant (1500 IU/15mL) + Von Willebrand factor human (1800 IU/15mL)Injection, powder, lyophilized, for solutionIntravenousInstituto Grifols, S.A.2014-07-08Not applicableItaly flag

Categories

ATC Codes
B02BD02 — Coagulation factor viii
Drug Categories
Chemical TaxonomyProvided by Classyfire
Description
Not Available
Kingdom
Organic Compounds
Super Class
Organic Acids
Class
Carboxylic Acids and Derivatives
Sub Class
Amino Acids, Peptides, and Analogues
Direct Parent
Peptides
Alternative Parents
Not Available
Substituents
Not Available
Molecular Framework
Not Available
External Descriptors
Not Available
Affected organisms
  • Humans and other mammals

Chemical Identifiers

UNII
P89DR4NY54
CAS number
139076-62-3

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. [Article]
  2. FDA Approved Drug Products: KOGENATE FS (Antihemophilic Factor [Recombinant], Formulated with Sucrose) For Intravenous Use, Lyophilized Powder for Reconstitution [Link]
  3. DailyMed Label: ADVATE [antihemophilic factor (recombinant)] lyophilized powder for reconstitution, for intravenous injection [Link]
UniProt
P00451
Genbank
M14113
PubChem Substance
46506209
RxNav
1300486
ChEMBL
CHEMBL2108175
PharmGKB
PA164750168
RxList
RxList Drug Page
Drugs.com
Drugs.com Drug Page
Wikipedia
Antihemophilic_Factor

Clinical Trials

Clinical Trials
Clinical Trial & Rare Diseases Add-on Data Package
Explore 4,000+ rare diseases, orphan drugs & condition pairs, clinical trial why stopped data, & more. Preview package
PhaseStatusPurposeConditionsCountStart DateWhy Stopped100+ additional columns
Not AvailableActive Not RecruitingNot AvailableHemophilia A1somestatusstop reasonjust information to hide
Not AvailableCompletedNot AvailableCoagulation Disorder / Hemophilia A1somestatusstop reasonjust information to hide
Not AvailableCompletedNot AvailableCongenital Factor VIII (FVIII) Deficiency / Hemophilia A1somestatusstop reasonjust information to hide
Not AvailableCompletedNot AvailableCoronavirus Disease 2019 (COVID‑19) / Hemophilia A1somestatusstop reasonjust information to hide
Not AvailableCompletedNot AvailableFactor VIII Deficiency / Hemophilia / Hemophilia A1somestatusstop reasonjust information to hide

Pharmacoeconomics

Manufacturers
Not Available
Packagers
  • Baxter International Inc.
  • Bayer Healthcare
  • CSL Behring LLC
  • GlaxoSmithKline Inc.
  • Grifols SA
  • Ipsen Pharmaceuticals Inc.
  • Octapharma USA
  • Talecris Biotherapeutics
  • Wyeth Pharmaceuticals
Dosage Forms
FormRouteStrength
Injection, powder, for solutionIntravenous1500 IU
Injection, powder, for solutionIntravenous; Parenteral100 UI
Injection, powder, for solutionIntravenous; Parenteral1500 UI
Injection, powder, lyophilized, for solution; kitIntravenous125 [iU]/1mL
Injection, powder, lyophilized, for solution; kitIntravenous250 [iU]/1mL
Injection, powder, lyophilized, for solution; kitIntravenous400 [iU]/1mL
Injection, powder, lyophilized, for solution; kitIntravenous500 [iU]/1mL
Injection, powder, lyophilized, for solution; kitIntravenous600 [iU]/1mL
Injection, powder, lyophilized, for solution; kitIntravenous750 [iU]/1mL
Injection, powder, lyophilized, for solution; kitIntravenous800 [iU]/1mL
KitIntravenous100 [iU]/1mL
KitIntravenous125 [iU]/1mL
KitIntravenous200 [iU]/1mL
KitIntravenous250 [iU]/1mL
KitIntravenous300 [iU]/1mL
KitIntravenous400 [iU]/1mL
KitIntravenous50 [iU]/1mL
KitIntravenous500 [iU]/1mL
KitIntravenous600 [iU]/1mL
KitIntravenous750 [iU]/1mL
KitIntravenous800 [iU]/1mL
Powder, for solutionIntravenous1500 unit / vial
Powder, for solutionIntravenous3000 unit / vial
SolutionIntravenous500.000 UI
Injection, powder, lyophilized, for solutionIntravenous1000 iu/5ml
Injection, powder, lyophilized, for solutionIntravenous1500 IU
Injection, powder, lyophilized, for solutionIntravenous1500 iu/5ml
Injection, powder, lyophilized, for solutionIntravenous2000 iu/5ml
Injection, powder, lyophilized, for solutionIntravenous2000 IU
Injection, powder, lyophilized, for solutionIntravenous250 iu/5ml
Injection, powder, lyophilized, for solutionIntravenous
Injection, powder, lyophilized, for solutionIntravenous500 iu/5ml
Injection, powder, lyophilized, for solution; kitIntravenous1000 [iU]/2mL
Injection, powder, lyophilized, for solution; kitIntravenous1500 [iU]/2mL
Injection, powder, lyophilized, for solution; kitIntravenous250 [iU]/2mL
Injection, powder, lyophilized, for solution; kitIntravenous500 [iU]/2mL
Injection, powder, lyophilized, for solution; kitIntravenous750 [iU]/5mL
Injection, powder, lyophilized, for solution; kitIntravenous750 [iU]/2mL
Injection, powder, for solutionIntravenous; Parenteral2500 UI
Injection, powder, lyophilized, for solutionParenteral
PowderParenteral1000 IU/10ml
PowderParenteral2000 IU/10ml
PowderParenteral250 IU/2.5ML
PowderParenteral500 IU/5ML
Injection, powder, lyophilized, for solutionIntravenous100 IU
Injection, powder, for solutionIntravenous; Parenteral4000 UI
Injection, powder, for solutionIntravenous; Parenteral5000 UI
Injection, powder, for solutionIntravenous; Parenteral6000 UI
Injection, powder, for solutionIntravenous; Parenteral750 UI
Injection, powder, for solutionIntravenous1000 IU/30mL
Injection, powder, lyophilized, for solutionIntravenous100 IU/10ml
Injection, powder, lyophilized, for solutionIntravenous1000 IU/10ml
Injection, powder, lyophilized, for solutionIntravenous250 IU/10ml
Injection, powder, lyophilized, for solutionIntravenous3000 IU/10ml
Injection, powder, lyophilized, for solutionIntravenous500 IU/10ml
Injection, solutionIntravenous100 IU
Injection, solutionIntravenous250 IU
Injection, solutionIntravenous500 IU
Powder, for solutionParenteral1000 U.I.
Powder, for solutionParenteral250 U.I.
Powder, for solutionParenteral500 U.I.
Injection, powder, for solutionParenteral5000 IU
Injection, powder, for solutionIntravenous100 IU
Injection, powder, for solutionIntravenous20 IU/mL
Injection, powder, for solutionIntravenous250 IU
Injection, solutionIntravenous100 IU/5ML
Injection, solutionIntravenous1000 IU/10ML
Injection, solutionIntravenous250 IU/10ML
Injection, solutionIntravenous500 IU/10ML
Injection, solutionIntravenous500 IU/20ML
Powder, for solutionParenteral250 UI
Powder, for solutionParenteral500 UI/10ML
Powder, for solutionIntravenous; Parenteral1000 UI
Powder, for solutionIntravenous; Parenteral250 UI
Powder, for solutionIntravenous; Parenteral500 UI
Injection, powder, lyophilized, for solutionIntravenous1000 IU
Powder, for solutionParenteral
Injection, powder, for solutionIntravenous3000 IU
Injection, powder, for solutionIntravenous; Parenteral1000 UI
Injection, powder, for solutionIntravenous; Parenteral250 UI
Injection, powder, for solutionIntravenous; Parenteral500 UI
PowderIntravenous2000 UI
Injection, powder, for solutionIntravenous3000 UI
Injection, powder, for solution
SolutionIntravenous500 UI
Injection, powder, for solutionIntravenous1000 UI
Injection, powder, for solutionIntravenous1000 IU
Injection, powder, for solutionIntravenous2000 IU
Injection, powder, for solutionIntravenous2000 UI
Injection, powder, for solutionIntravenous250 UI
Injection, powder, for solutionIntravenous500 UI
Injection, powder, for solutionIntravenous500 IU
PowderIntravenous3000 UI
KitIntravenous1000 [iU]/2.5mL
KitIntravenous2000 [iU]/5mL
KitIntravenous250 [iU]/2.5mL
KitIntravenous3000 [iU]/5mL
KitIntravenous500 [iU]/2.5mL
Powder, for solutionIntravenous2000 unit / vial
Injection, powder, lyophilized, for solutionIntravenous1000 IU/vial
Injection, powder, lyophilized, for solutionIntravenous250 IU/vial
Injection, powder, lyophilized, for solutionIntravenous500 IU/vial
Injection, powder, for solutionIntravenous; Parenteral2000 UI
Injection, powder, for solutionIntravenous; Parenteral3000 UI
Injection, powder, lyophilized, for solution; kitIntravenous1000 [iU]/2.5mL
Injection, powder, lyophilized, for solution; kitIntravenous250 [iU]/2.5mL
Injection, powder, lyophilized, for solution; kitIntravenous3000 [iU]/5mL
Injection, powder, lyophilized, for solution; kitIntravenous500 [iU]/2.5mL
Kit; powder, for solutionIntravenous1000 unit / vial
Kit; powder, for solutionIntravenous2000 unit / vial
Kit; powder, for solutionIntravenous250 unit / vial
Kit; powder, for solutionIntravenous3000 unit / vial
Kit; powder, for solutionIntravenous500 unit / vial
SolutionIntravenous2000 UI
Injection, powder, lyophilized, for solutionIntravenous250 IU
PowderIntravenous1000 IU
PowderIntravenous1500 IU
PowderIntravenous250 IU
Injection, powder, lyophilized, for solution; kitIntravenous375 [iU]/1mL
Injection, powder, lyophilized, for solution; kitIntravenous62.5 [iU]/1mL
Injection, powder, for solutionIntravenous; Parenteral1000 IU
Injection, powder, for solutionIntravenous; Parenteral2000 IU
Injection, powder, for solutionIntravenous; Parenteral250 IU
Injection, powder, for solutionIntravenous; Parenteral500 IU
Injection, powder, for solutionParenteral50 IU/ML
Injection, powder, lyophilized, for solutionIntravenous
Injection, powder, for solutionIntravenous1000 IU/10ML
Injection, powder, for solutionIntravenous250 IU/10ML
Injection, powder, for solutionIntravenous500 IU/10ML
Injection, powder, for solutionParenteral1000 U.I./10ML
Injection, powder, for solutionParenteral1000 U.I./5ML
Injection, powder, for solutionParenteral250 U.I./5ML
Injection, powder, for solutionParenteral250 U.I./10ML
Injection, powder, for solutionParenteral500 U.I./10ML
Injection, powder, for solutionParenteral500 U.I./5ML
Injection, powder, lyophilized, for solution; kitIntravenous100 [iU]/1mL
Injection, powder, lyophilized, for solution; kitIntravenous1000 [iU]/5mL
Injection, powder, lyophilized, for solution; kitIntravenous1500 [iU]/5mL
Injection, powder, lyophilized, for solution; kitIntravenous2000 [iU]/5mL
Injection, powder, lyophilized, for solution; kitIntravenous25 [iU]/1mL
Injection, powder, lyophilized, for solution; kitIntravenous250 [iU]/5mL
Injection, powder, lyophilized, for solution; kitIntravenous50 [iU]/1mL
Injection, powder, lyophilized, for solution; kitIntravenous500 [iU]/5mL
SolutionIntravenous
Powder, for solutionIntravenous1000 unit / vial
Powder, for solutionIntravenous250 unit / vial
Powder, for solutionIntravenous500 unit / vial
InjectionIntravenous1000 IU/4ml
InjectionIntravenous2000 IU/4ml
InjectionIntravenous3000 IU/4ml
InjectionIntravenous500 IU/4ml
SolutionIntravenous250 IU/4ml
Injection, powder, for solutionParenteral
Injection, powder, for solutionIntravenous2500 UI
Injection, powder, for solutionIntravenous4000 UI
PowderIntravenous500 IU
Injection, powder, lyophilized, for solutionIntravenous500 IU
Injection, solutionIntravenous
Injection, powder, for solutionIntravenous1000 iu/1vial
Injection, powder, for solutionIntravenous250 iu/1vial
Injection, powder, for solutionIntravenous500 iu/1vial
Injection, powder, for solution1000 IU
Injection, powder, for solution1500 IU
Injection, powder, for solution250 IU
Injection, powder, for solution500 IU
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
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Patents
Patent NumberPediatric ExtensionApprovedExpires (estimated)Region
CA2124690No2007-09-112013-10-01Canada flag
CA1339477No1997-09-232014-09-23Canada flag

Properties

State
Solid
Experimental Properties
PropertyValueSource
hydrophobicity-0.533Not Available
isoelectric point6.97Not Available

Targets

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Kind
Protein
Organism
Humans
Pharmacological action
Yes
Actions
Activator
General 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. Factor Xa activates pro-inflammatory signaling pathways in a protease-activated receptor (PAR)-dependent manner (PubMed:24041930, PubMed:30568593, PubMed:34831181). Up-regulates expression of protease-activated receptors (PARs) F2R, F2RL1 and F2RL2 in dermal microvascular endothelial cells (PubMed:35738824). Triggers the production of pro-inflammatory cytokines, such as MCP-1/CCL2 and IL6, in cardiac fibroblasts and umbilical vein endothelial cells in PAR-1 (F2R)-dependent manner (PubMed:30568593, PubMed:34831181). Triggers the production of pro-inflammatory cytokines, such as MCP-1/CCL2, IL6, TNF-alpha/TNF, IL-1beta/IL1B, IL8/CXCL8 and IL18, in endothelial cells and atrial tissues (PubMed:24041930, PubMed:35738824, PubMed:9780208). Induces expression of adhesion molecules, such as ICAM1, VCAM1 and SELE, in endothelial cells and atrial tissues (PubMed:24041930, PubMed:35738824, PubMed:9780208). Increases expression of phosphorylated ERK1/2 in dermal microvascular endothelial cells and atrial tissues (PubMed:24041930, PubMed:35738824). Triggers activation of the transcription factor NF-kappa-B in dermal microvascular endothelial cells and atrial tissues (PubMed:24041930, PubMed:35738824). Up-regulates expression of plasminogen activator inhibitor 1 (SERPINE1) in atrial tissues (PubMed:24041930)
Specific Function
calcium ion binding
Gene Name
F10
Uniprot ID
P00742
Uniprot Name
Coagulation factor X
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. [Article]
  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. [Article]
  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. [Article]
  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. [Article]
  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. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Yes
Actions
Cofactor
General 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
Specific Function
calcium ion binding
Gene Name
F9
Uniprot ID
P00740
Uniprot Name
Coagulation factor IX
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. [Article]
  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. [Article]
  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. [Article]
  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. [Article]
  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. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Yes
Actions
Binder
General 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 clearance from plasma
Specific Function
collagen binding
Gene Name
VWF
Uniprot ID
P04275
Uniprot Name
von Willebrand factor
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. [Article]
  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. [Article]
  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. [Article]
  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. [Article]
  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. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Unknown
Actions
Antagonist
General Function
Catalyzes the 2-hydroxylation of not only racemic phytanoyl-CoA and the isomers of 3-methylhexadecanoyl-CoA, but also a variety of other mono-branched 3-methylacyl-CoA esters (with a chain length of at least seven carbon atoms) and straight-chain acyl-CoA esters (with a chain length longer than four carbon atoms) (PubMed:10744784, PubMed:12031666, PubMed:12923223, PubMed:9326939). Does not hydroxylate long and very long straight chain acyl-CoAs or 2-methyl- and 4-methyl-branched acyl-CoAs (PubMed:10744784, PubMed:12923223)
Specific Function
carboxylic acid binding
Gene Name
PHYH
Uniprot ID
O14832
Uniprot Name
Phytanoyl-CoA dioxygenase, peroxisomal
Molecular Weight
38538.065 Da
References
  1. 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. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Unknown
Actions
Binder
General 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 then returns to the cell membrane surface
Specific Function
asialoglycoprotein receptor activity
Gene Name
ASGR2
Uniprot ID
P07307
Uniprot Name
Asialoglycoprotein receptor 2
Molecular Weight
35092.04 Da
References
  1. 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. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Unknown
Actions
Chaperone
General Function
Endoplasmic reticulum chaperone that plays a key role in protein folding and quality control in the endoplasmic reticulum lumen (PubMed:2294010, PubMed:23769672, PubMed:23990668, PubMed:28332555). Involved in the correct folding of proteins and degradation of misfolded proteins via its interaction with DNAJC10/ERdj5, probably to facilitate the release of DNAJC10/ERdj5 from its substrate (By similarity). Acts as a key repressor of the ERN1/IRE1-mediated unfolded protein response (UPR) (PubMed:1550958, PubMed:19538957). In the unstressed endoplasmic reticulum, recruited by DNAJB9/ERdj4 to the luminal region of ERN1/IRE1, leading to disrupt the dimerization of ERN1/IRE1, thereby inactivating ERN1/IRE1 (By similarity). Accumulation of misfolded protein in the endoplasmic reticulum causes release of HSPA5/BiP from ERN1/IRE1, allowing homodimerization and subsequent activation of ERN1/IRE1 (By similarity). Plays an auxiliary role in post-translational transport of small presecretory proteins across endoplasmic reticulum (ER). May function as an allosteric modulator for SEC61 channel-forming translocon complex, likely cooperating with SEC62 to enable the productive insertion of these precursors into SEC61 channel. Appears to specifically regulate translocation of precursors having inhibitory residues in their mature region that weaken channel gating. May also play a role in apoptosis and cell proliferation (PubMed:26045166)
Specific Function
ATP binding
Gene Name
HSPA5
Uniprot ID
P11021
Uniprot Name
Endoplasmic reticulum chaperone BiP
Molecular Weight
72332.425 Da
References
  1. 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. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Unknown
Actions
Chaperone
General 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 (PubMed:7876246). Interacts with the DNA-binding domain of NR3C1 and mediates its nuclear export (PubMed:11149926). Involved in maternal gene expression regulation. May participate in oocyte maturation via the regulation of calcium homeostasis (By similarity). Present in the cortical granules of non-activated oocytes, is exocytosed during the cortical reaction in response to oocyte activation and might participate in the block to polyspermy (By similarity)
Specific Function
calcium ion binding
Gene Name
CALR
Uniprot ID
P27797
Uniprot Name
Calreticulin
Molecular Weight
48141.2 Da
References
  1. 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. [Article]
  2. 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. [Article]
  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. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Unknown
Actions
Chaperone
General Function
Calcium-binding protein that interacts with newly synthesized monoglucosylated 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 that escape the ER of immature thymocytes, it may function as a signaling complex regulating thymocyte maturation. Additionally it may play a role in receptor-mediated endocytosis at the synapse
Specific Function
calcium ion binding
Gene Name
CANX
Uniprot ID
P27824
Uniprot Name
Calnexin
Molecular Weight
67567.695 Da
References
  1. 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. [Article]
  2. 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. [Article]
  3. 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. [Article]
  4. 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. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Unknown
Actions
Chaperone
General 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
Specific Function
D-mannose binding
Gene Name
LMAN1
Uniprot ID
P49257
Uniprot Name
Protein ERGIC-53
Molecular Weight
57548.665 Da
References
  1. 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. [Article]
  2. 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. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Unknown
Actions
Modulator
General Function
Endocytic receptor involved in endocytosis and in phagocytosis of apoptotic cells (PubMed:11907044, PubMed:12713657). Required for early embryonic development (By similarity). 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. Acts as an LRPAP1 alpha-2-macroglobulin receptor (PubMed:1702392, PubMed:26142438). Acts as TAU/MAPT receptor and controls the endocytosis of TAU/MAPT as well as its subsequent spread (PubMed:32296178). May modulate cellular events, such as APP metabolism, kinase-dependent intracellular signaling, neuronal calcium signaling as well as neurotransmission (PubMed:12888553)
Specific Function
alpha-2 macroglobulin receptor activity
Gene Name
LRP1
Uniprot ID
Q07954
Uniprot Name
Prolow-density lipoprotein receptor-related protein 1
Molecular Weight
504601.695 Da
References
  1. 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. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Unknown
Actions
Modulator
General 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
Specific Function
calcium ion binding
Gene Name
MCFD2
Uniprot ID
Q8NI22
Uniprot Name
Multiple coagulation factor deficiency protein 2
Molecular Weight
16390.175 Da
References
  1. 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. [Article]

Enzymes

Kind
Protein
Organism
Humans
Pharmacological action
Unknown
Actions
Activator
General 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. Thrombin triggers the production of pro-inflammatory cytokines, such as MCP-1/CCL2 and IL8/CXCL8, in endothelial cells (PubMed:30568593, PubMed:9780208)
Specific Function
calcium ion binding
Gene Name
F2
Uniprot ID
P00734
Uniprot Name
Prothrombin
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. [Article]
  2. Ratnoff OD, Lewis JH: Heckathorn's disease: variable functional dificiency of antihemophilic factor (factor VIII). Blood. 1975 Aug;46(2):161-73. [Article]
  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. [Article]
  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. [Article]
  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. [Article]
Kind
Protein
Organism
Humans
Pharmacological action
Unknown
Actions
Substrate
General 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)
Specific Function
calcium ion binding
Gene Name
PROC
Uniprot ID
P04070
Uniprot Name
Vitamin K-dependent protein C
Molecular Weight
52070.82 Da
References
  1. 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. [Article]

Drug created at June 13, 2005 13:24 / Updated at October 17, 2024 13:59