coagulation factor VIII

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

   GtoImmuPdb view: OFF :     Currently no data for coagulation factor VIII in GtoImmuPdb

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
FVIII | HEMA | coagulation factor VIII, procoagulant component
Database Links
CATH/Gene3D
ChEMBL Target
DrugBank Target
Ensembl Gene
Entrez Gene
GenitoUrinary Development Molecular Anatomy Project
Human Protein Atlas
KEGG Gene
OMIM
Orphanet
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:  8

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

Antagonists
Key to terms and symbols Click column headers to sort
Ligand Sp. Action Affinity Units Reference
drotrecogin alfa Hs Inhibition - - 6-7
Antithrombotic effect thought to occur via inhibition of factors Va and VIIIa [6-7]
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
Drotrecogin alfa (activated recombinant protein kinase C) was initially seen as a breakthough in the treatment of severe sepsis [3] with the results of initial clinical trials having positive outcomes [1]. However, the beneficial effects of this drug are now disputed [2,4-5].

References

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1. Bernard GR, Vincent JL, Laterre PF, LaRosa SP, Dhainaut JF, Lopez-Rodriguez A, Steingrub JS, Garber GE, Helterbrand JD, Ely EW et al.. (2001) Efficacy and safety of recombinant human activated protein C for severe sepsis. N. Engl. J. Med.344 (10): 699-709. [PMID:11236773]

2. Brun-Buisson C. (2012) ACP Journal Club: drotrecogin alfa (activated) did not reduce mortality at 28 or 90 days in patients with septic shock. Ann. Intern. Med.157 (4): JC2-11. [PMID:22910965]

3. Grinnell BW, Joyce D. (2001) Recombinant human activated protein C: a system modulator of vascular function for treatment of severe sepsis. Crit. Care Med.29 (7 Suppl): S53-60; discussion S60-1. [PMID:11445735]

4. Holder AL, Huang DT. (2013) A dream deferred: the rise and fall of recombinant activated protein C. Crit Care17 (2): 309 [Epub ahead of print]. [PMID:23509922]

5. Kalil AC, Florescu DF. (2013) Severe sepsis: are PROWESS and PROWESS-SHOCK trials comparable? A clinical and statistical heterogeneity analysis. Crit Care17 (4): 167 [Epub ahead of print]. [PMID:23826709]

6. Kanji S, Devlin JW, Piekos KA, Racine E. (2001) Recombinant human activated protein C, drotrecogin alfa (activated): a novel therapy for severe sepsis. Pharmacotherapy21 (11): 1389-402. [PMID:11714212]

7. 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]

8. 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. Structure16 (4): 597-606. [PMID:18400180]

How to cite this page

Blood coagulation components: coagulation factor VIII. Last modified on 03/07/2016. Accessed on 22/10/2017. IUPHAR/BPS Guide to PHARMACOLOGY, http://www.guidetopharmacology.org/GRAC/ObjectDisplayForward?objectId=2607.