endoplasmic reticulum to Golgi vesicle-mediated transport
COPII vesicle coating
acute-phase response
blood coagulation, intrinsic pathway
platelet activation
Sources:Amigo / QuickGO
Orthologs
Species
Human
Mouse
Entrez
2157
14069
Ensembl
ENSG00000185010
ENSMUSG00000031196
UniProt
P00451
Q06194
RefSeq (mRNA)
NM_000132 NM_019863
NM_001161373 NM_001161374 NM_007977
RefSeq (protein)
NP_000123 NP_063916
NP_001154845 NP_001154846 NP_032003
Location (UCSC)
Chr X: 154.84 – 155.03 Mb
Chr X: 74.22 – 74.43 Mb
PubMed search
[3]
[4]
Wikidata
View/Edit Human
View/Edit Mouse
Coagulation factor VIII (Factor VIII, FVIII, also known as anti-hemophilic factor (AHF)) is an essential blood clotting protein. In humans, it is encoded by F8 gene.[5][6] Defects in this gene result in hemophilia A, an X-linked bleeding disorder.[7]
Factor VIII is produced in the liver's sinusoidal cells and endothelial cells outside the liver throughout the body. This protein circulates in the bloodstream in an inactive form, bound to another molecule called von Willebrand factor, until an injury that damages blood vessels occurs.[8] In response to injury, coagulation factor VIII is activated and separates from von Willebrand factor. The active protein (sometimes written as coagulation factor VIIIa) interacts with another coagulation factor called factor IX. This interaction sets off a chain of additional chemical reactions that form a blood clot.[8]
Factor VIII participates in blood coagulation; it is a cofactor for factor IXa, which, in the presence of Ca2+ and phospholipids, forms a complex that converts factor X to the activated form Xa. The factor VIII gene produces two alternatively spliced transcripts. Transcript variant 1 encodes a large glycoprotein, isoform a, which circulates in plasma and associates with von Willebrand factor in a noncovalent complex. This protein undergoes multiple cleavage events. Transcript variant 2 encodes a putative small protein, isoform b, which consists primarily of the phospholipid binding domain of factor VIIIc. This binding domain is essential for coagulant activity.[9]
People with high levels of factor VIII are at increased risk for deep vein thrombosis and pulmonary embolism.[10] Copper is a required cofactor for factor VIII and copper deficiency is known to increase the activity of factor VIII.[11]
Factor VIII is available as a medication that is on the WHO Model List of Essential Medicines, the most important medications needed in a basic health system.[12]
^ abcGRCh38: Ensembl release 89: ENSG00000185010 – Ensembl, May 2017
^ abcGRCm38: Ensembl release 89: ENSMUSG00000031196 – Ensembl, May 2017
^"Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^"Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^Toole JJ, Knopf JL, Wozney JM, Sultzman LA, Buecker JL, Pittman DD, Kaufman RJ, Brown E, Shoemaker C, Orr EC (1984). "Molecular cloning of a cDNA encoding human antihaemophilic factor". Nature. 312 (5992): 342–47. Bibcode:1984Natur.312..342T. doi:10.1038/312342a0. PMID 6438528. S2CID 4313575.
^Truett MA, Blacher R, Burke RL, Caput D, Chu C, Dina D, Hartog K, Kuo CH, Masiarz FR, Merryweather JP (October 1985). "Characterization of the polypeptide composition of human factor VIII:C and the nucleotide sequence and expression of the human kidney cDNA". DNA. 4 (5): 333–49. doi:10.1089/dna.1985.4.333. PMID 3935400.
^Antonarakis SE (July 1995). "Molecular genetics of coagulation factor VIII gene and hemophilia A". Thrombosis and Haemostasis. 74 (1): 322–28. doi:10.1055/s-0038-1642697. PMID 8578479. S2CID 23435953.
^ ab"NIH: F8 – coagulation factor VIII". National Institutes of Health.
^Jenkins PV, Rawley O, Smith OP, O'Donnell JS (June 2012). "Elevated factor VIII levels and risk of venous thrombosis". British Journal of Haematology. 157 (6): 653–63. doi:10.1111/j.1365-2141.2012.09134.x. PMID 22530883.
^Milne DB, Nielsen FH (March 1996). "Effects of a diet low in copper on copper-status indicators in postmenopausal women". The American Journal of Clinical Nutrition. 63 (3): 358–64. doi:10.1093/ajcn/63.3.358. PMID 8602593.
^"19th WHO Model List of Essential Medicines" (PDF). WHO. April 2015. Retrieved May 10, 2015.
Coagulation factorVIII (FactorVIII, FVIII, also known as anti-hemophilic factor (AHF)) is an essential blood clotting protein. In humans, it is encoded...
a blood clotting disorder caused by a genetic deficiency in clotting factorVIII, thereby resulting in significant susceptibility to bleeding, both internally...
function; elements of note are: the D'/D3 domain, which binds to factorVIII (von Willebrand factor type D domain). the A1 domain, which binds to: platelet GPIb-receptor...
best-known coagulation factor disorders are the hemophilias. The three main forms are hemophilia A (factorVIII deficiency), hemophilia B (factor IX deficiency...
occurs due to low amounts of clotting factorVIII, and haemophilia B, which occurs due to low levels of clotting factor IX. They are typically inherited from...
haemophilia were principally infected via the plasma-derived product known as factorVIII, a processed pharmaceutical product sourced from the United States and...
inherited mutation of the gene for factor IX, and resulting in a deficiency of factor IX. It is less common than factorVIII deficiency (haemophilia A). Haemophilia...
agglutination (RIPA) assays. FactorVIII levels are also performed because factorVIII is bound to VWF which protects the factorVIII from rapid breakdown within...
factor IX with its cofactor, factorVIII in a complex known as intrinsic pathway; and factor VII with its cofactor, tissue factor in a complex known as extrinsic...
made of a virus (AAV5) that has been modified to contain the gene for factorVIII, which is lacking in people with hemophilia A. It is an adeno-associated...
Along with the purchase of Cutter, Bayer acquired Cutter's FactorVIII business. FactorVIII, a clotting agent used to treat hemophilia, was produced,...
against coagulation factorVIII. These autoantibodies constitute the most common spontaneous inhibitor to any coagulation factor and may induce spontaneous...
is factorVIII (also called antihaemophilic factor or AHF), which is why cryoprecipitate is sometimes called cryoprecipitated antihaemophilic factor or...
factor. If the patient has a high titer antibody against factorVIII, try porcine factorVIII or prothrombin complex concentrates to stop the bleeding...
factorVIII, a blood-clotting protein that is administered to patients with the bleeding disorder hemophilia, who are unable to produce factorVIII in...
1985. Hemophilia A causes a deficiency in FactorVIII, a protein required for blood clotting. FactorVIII injections are a common treatment to prevent...
is a medication used for the treatment of hemophilia A (congenital factorVIII deficiency). Efanesoctocog alfa was approved for medical use in the United...
deficiency of FactorVIII causes classic hemophilia A while deficiencies of Factor IX cause "Christmas disease"(hemophilia B). Antibodies to FactorVIII can also...
coagulation factor IX and to factor X, mediating the activation of the latter. This is normally the function of coagulation factorVIII, which is missing...
associated with reduced levels of von Willebrand factor – because of increased clearance – and factorVIII, which is related to thrombotic risk . A number...
into factor IXa, in the presence of Ca2+, membrane phospholipids, and a FactorVIII cofactor, it hydrolyses one arginine-isoleucine bond in factor X to...
As a haemophiliac, he became infected with HIV from a contaminated factorVIII blood treatment and, when diagnosed in December 1984, was given six months...
Recombinant factor VIIa, which is an activated form of factor VII, bypasses factorsVIII and IX and causes coagulation without the need for factorsVIII and IX...
following coagulation factors: I (fibrinogen), II (prothrombin), V (proaccelerin), VIII (anti-hemophilic factor), X (Stuart–Prower factor), XI (plasma thromboplastin...
HIV-infected infusions of FactorVIII. Ricky, Robert, and Randy were hemophiliacs who contracted HIV from infusions of FactorVIII when they were less than...
Intrinsic tenase complex contains the active factor IX (IXa), its cofactor factorVIII (VIIIa), the substrate (factor X), and they are activated by negatively...
recombinant coagulation factorVIII since it has an amino acid sequence which compares to the 90 + 80 kDa form of factorVIII (BDDrFVIII). It also has...
list of Alboran Sea; see List of NATO country codes A brand name of factorVIII (medication) Y8 (disambiguation) This disambiguation page lists articles...
factorVIII concentrate Berinin P, freeze-dried human coagulation factor IX concentrate Factor X P Behring, a freeze-dried human coagulation factor IX...
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