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coagulation factor VIII

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Target id: 2607

Nomenclature: coagulation factor VIII

Family: Blood coagulation components

Gene and Protein Information Click here for help
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human - 2351 Xq28 F8 coagulation factor VIII
Mouse - 2319 X 38.17 cM F8 coagulation factor VIII
Rat - - 18p13 F8 coagulation factor VIII
Previous and Unofficial Names Click here for help
coagulation factor VIII, procoagulant component | FVIII | HEMA
Database Links Click here for help
Alphafold
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 Click here for help
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

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Inhibitors
Key to terms and symbols Click column headers to sort
Ligand Sp. Action Value Parameter Reference
drotrecogin alfa Peptide Approved drug Primary target of this compound Click here for species-specific activity table Hs Inhibition - - 1-2
Antithrombotic effect thought to occur via inhibition of factors Va and VIIIa [1-2]
Immuno Process Associations
Immuno Process:  Inflammation
Clinically-Relevant Mutations and Pathophysiology Click here for help
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. A long-acting recombinant FVIII-Fc fusion protein (efanesoctocog alfa; ALTUVIIIO®) has been approved as a treatment for hemophilia A.

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]

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