Coagulation Factor VIII Active Protein | F8 active protein
Human Coagulation Factor VIII
Sterile Filtered White lyophilized (freeze-dried) powder.
NCBI and Uniprot Product Information
Uniprot Description
F8: Factor VIII, along with calcium and phospholipid, acts as a cofactor for factor IXa when it converts factor X to the activated form, factor Xa. Defects in F8 are the cause of hemophilia A (HEMA). 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. Of particular interest for the understanding of the function of F8 is the category of CRM (cross-reacting material) positive patients (approximately 5%) that have considerable amount of F8 in their plasma (at least 30% of normal), but the protein is non- functional; i.e. the F8 activity is much less than the plasma protein level. CRM-reduced is another category of patients in which the F8C antigen and activity are reduced to approximately the same level. Most mutations are CRM negative, and probably affect the folding and stability of the protein. Belongs to the multicopper oxidase family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: Xq28
Cellular Component: endoplasmic reticulum lumen; ER to Golgi transport vesicle; ER-Golgi intermediate compartment membrane; extracellular region; plasma membrane
Molecular Function: protein binding
Biological Process: blood coagulation; blood coagulation, intrinsic pathway; COPII coating of Golgi vesicle; ER to Golgi vesicle-mediated transport; platelet degranulation
Disease: Hemophilia A
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Product Notes
The F8 f8 (Catalog #AAA142873) is an Active Protein produced from Human Plasma and is intended for research purposes only. The product is available for immediate purchase. It is sometimes possible for the material contained within the vial of "Coagulation Factor VIII, Active Protein" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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