Anticorps Factor VIII (GMA-8011): sc-65950

Factor VIII is a glycoprotein cofactor that serves as a critical component in the blood coagulation pathway. Insufficient expression levels or expression of nonfunctional Factor VIII results in hemophilia A, a common severe hereditary bleeding disorder. In the liver, the main site of factor VIII synthesis, the mature polypetide chain of 2332 amino acids is secreted into the lumen of the endoplasmic reticulum, where it interacts with various chaperone proteins, including Calreticulin, Calnexin and IgG-binding protein. From the lumen, a portion of Factor VIII translocates to the Golgi and undergoes activation via proteolysis of both the heavy and light chain portions of the protein into three fragments. Finally, proteolysis of activated Factor VIII by Factor Xa, Protein C or Thrombin results in inactivation of Factor VIII. Survival of Factor VIII in the bloodstream requires binding to von Willebrand factor (VWF) at both the amino and carboxy termini of the light chain. Point mutations occuring in those binding domains as well as at other active sites of Factor VIII likely underly 90-95% of disease cases.

L'anticorps Factor VIII préféré est l'Anticorps Factor VIII (R8B12): sc-73597.