coagulation factor VIII | Blood coagulation components | IUPHAR/BPS Guide to PHARMACOLOGY

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coagulation factor VIII

Target not currently curated in GtoImmuPdb

Target id: 2607

Nomenclature: coagulation factor VIII

Family: Blood coagulation components

Annotation status:  image of a grey circle Awaiting annotation/under development. Please contact us if you can help with annotation.  » Email us

Gene and Protein Information
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human - 2351 Xq28 F8 coagulation factor VIII
Mouse - 2319 X A7-B F8 coagulation factor VIII
Rat - - 18p13 F8 coagulation factor VIII
Previous and Unofficial Names
coagulation factor VIII, procoagulant component | FVIII | HEMA
Database Links
CATH/Gene3D
ChEMBL Target
DrugBank Target
Ensembl Gene
Entrez Gene
Human Protein Atlas
KEGG Gene
OMIM
Orphanet
Pharos
RefSeq Nucleotide
RefSeq Protein
UniProtKB
Wikipedia
Selected 3D Structures
Image of receptor 3D structure from RCSB PDB
Description:  Crystal structure of human factor VIII: implications for the formation of the factor IXa-factor VIIIa complex.
PDB Id:  3CDZ
Resolution:  3.98Å
Species:  Human
References:  3

Download all structure-activity data for this target as a CSV file

Inhibitors
Key to terms and symbols Click column headers to sort
Ligand Sp. Action Value Parameter Reference
drotrecogin alfa Hs Inhibition - - 1-2
Antithrombotic effect thought to occur via inhibition of factors Va and VIIIa [1-2]
Immuno Process Associations
Immuno Process:  Inflammation
GO Annotations:  Associated to 1 GO processes, IEA only
click arrow to show/hide IEA associations
GO:0006953 acute-phase response IEA
Clinically-Relevant Mutations and Pathophysiology
Disease:  Factor VIII deficiency
Disease Ontology: DOID:12134
OMIM: 134500
Disease:  Mild hemophilia A
Synonyms: Hemophilia A; HEMA [OMIM: 306700]
OMIM: 306700
Orphanet: ORPHA169808
Disease:  Moderately severe hemophilia A
Synonyms: Hemophilia A; HEMA [OMIM: 306700]
OMIM: 306700
Orphanet: ORPHA169805
Disease:  Severe hemophilia A
Synonyms: Hemophilia A; HEMA [OMIM: 306700]
OMIM: 306700
Orphanet: ORPHA169802
Disease:  Symptomatic form of hemophilia A in female carriers
Orphanet: ORPHA177926
General Comments
Recombinant factor VIII (Voncento®) is used to treat hemophilia A and von Willebrand diseases.

References

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1. Kanji S, Devlin JW, Piekos KA, Racine E. (2001) Recombinant human activated protein C, drotrecogin alfa (activated): a novel therapy for severe sepsis. Pharmacotherapy, 21 (11): 1389-402. [PMID:11714212]

2. Kapur S, Kupfer Y, Tessler S. (2001) Recombinant human activated protein C for severe sepsis. N. Engl. J. Med., 345 (3): 219-20; author reply 220-1. [PMID:11463021]

3. Ngo JC, Huang M, Roth DA, Furie BC, Furie B. (2008) Crystal structure of human factor VIII: implications for the formation of the factor IXa-factor VIIIa complex. Structure, 16 (4): 597-606. [PMID:18400180]

How to cite this page

Blood coagulation components: coagulation factor VIII. Last modified on 24/01/2019. Accessed on 15/07/2020. IUPHAR/BPS Guide to PHARMACOLOGY, http://www.guidetopharmacology.org/GRAC/ObjectDisplayForward?objectId=2607.