In a galaxy far, far away… or in blood vessels far, far away awaits a powerful superhero Factor VIII. Yea, when this guy detects damage to his known territory, which is the entire blood vessel galaxy, he is out to risk his life to maintain the balance of the Hemostasis Empire. This superhero never sleeps. He is in constant circulation, circulating the blood vessels of the human body. Factor VIII though does not work alone when circulating the blood vessels when in inactive form. Factor VIII and his sidekick vWF both are bound together looking for damage and awaiting for injury that may arise at any second. When injury happens (Intrinsic Pathway) vWF and Factor VIII splits. Factor VIII goes into action (going …show more content…
He particularly does not have a hide out because he is always patrolling with all eyes wide open. Factor VIII’s headquarters or base is all over, circulating blood vessels. Factor VIII was born from the edge of the galaxy, from the sub endothelial cells. “Factor VIII is synthesized by various tissues, including liver, kidney, and spleen, as an inactive single-chain protein. After extensive posttranslational processing, factor VIII is released into the circulation as a set of heterodimeric proteins. This heterogenous population of factor VIII molecules readily interacts with vWF, which is produced and secreted by vascular endothelial cells (bloodjournal.org).” Factor VIII’s side kick, vWF, is also from the edge of the galaxy, the sub endothelial …show more content…
In addition, there are some hemostasis empires out there who do not have VIII patrolling their galaxy. Hemophilia A is a big problem. This is a factor VIII deficiency (X-linked) which is a genetic disorder caused by missing or defective factor VIII. It is known to be passed down from parents to children, however, about 1/3 of cases are caused by a spontaneous mutation or a change in a gene. “According to the US Centers for Disease Control and Prevention, hemophilia occurs in approximately 1 in 5,000 live births. There are about 20,000 people with hemophilia in the US. All races and ethnic groups are affected. Hemophilia A is four times as common as hemophilia B while more than half of patients with hemophilia A have the severe form of hemophilia (hemophilia.org).” Symptoms of people with hemophilia A often, bleed longer than other people. Bleeds can occur internally, into joints and muscles, or externally, from minor cuts. How frequently a person bleeds and the severity of those bleeds depends on how much FVIII is in the plasma, the straw-colored fluid portion of blood.
Anti-C2 antibodies is an archenemy of the superhero. This antibody disrupt the binding of FVIII to phospholipid and vWF. Also there are antibodies to A2 and A3 interfere with FVIII binding to factor X and factor IXa. So there are alloantibodies out there circulating the galaxy that holds Factor VIII hostage. These though are very rare,