Factor VIII ist ein Gen, das durch das Symbol F8 kodiert wird. Andere Namen sind: Coagulation Antihemophilic factor; AHF; Procoagulant component; F8; F8C. Factor VIII hat eine Masse von 267.01kDa, eine Aminosäurelänge von 2351, und ist an Hemophilia A beteiligt.
Wir bieten 12 antikörper gegen Factor VIII, aufgewachsen in Kaninchen, Maus und Human, welche geeignet sind für WB, ELISA, IP and RIA mit Proben abgeleitet von Human und Schwein.
Gen- und Proteininformationen
UniProt Zusammenfassung
Factor VIII, along with calcium and phospholipid, acts as a cofactor for F9/factor IXa when it converts F10/factor X to the activated form, factor Xa.
Entrez Zusammenfassung
This gene encodes coagulation factor VIII, which participates in the intrinsic pathway of blood coagulation; factor VIII is a cofactor for factor IXa which, in the presence of Ca+2 and phospholipids, converts factor X to the activated form Xa. This 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. Defects in this gene results in hemophilia A, a common recessive X-linked coagulation disorder.
Rolle bei Krankheiten
Hemophilia A: A disorder of blood coagulation characterized by a permanent tendency to hemorrhage. About 50% of patients have severe hemophilia resulting in frequent spontaneous bleeding into joints, muscles and internal organs. Less severe forms are characterized by bleeding after trauma or surgery.
Sequenzähnlichkeiten
Belongs to the multicopper oxidase family.
Posttranslationale Modifikation
Sulfation on Tyr-1699 is essential for binding vWF.
Zellort
Secreted > Extracellular space.
