Full length protein corresponding to aa1-216 from human Factor VIII.
Von Willebrand factor (VWF) is a blood glycoprotein required for normal hemostasis. VWF mediates the adhesion of platelets to sites of vascular damage by binding to specific platelet membrane glycoproteins and to constituents of exposed connective tissue. It also induces expression of the fibrinogen receptor and thus initiates platelet-platelet interaction. It is a carrier protein for blood clotting factor VIII and also stabilizes factor VIII and may serve to transport it to sites of vessel injury. It is assembled from identical approximately 250kD subunits into disulfide-linked multimers that may be greater 20,000kD. Mutations in VWD disrupt the complex biosynthetic process at several steps to impair the assembly, intracellular targeting, or secretion of VWF multimers. Deficiency of VWF leads to von Willebrand disease (VWD), and a large number of other diseases, including thrombotic thrombocytopenic purpura, Heydes syndrome, and possibly hemolytic-uremic syndrome. It is mapped on human chromosome 12p13.3. Applications: Suitable for use in Western Blot. Other applications have not been tested. Recommended Dilutions: Optimal dilutions to be determined by the researcher. Amino Acid Sequence: MRIQDPGKVFFGNVDSSGIKHNIFNPPIIARYIRLHPTHYSIRSTLRMELMGCDLNSCSMPLGMESKAISDAQITASSYFTNMFATWSPSKARLHLQGRSNAWRPQVNNPKEWLQVDFQKTMKVTGVTTQGVKSLLTSMYVKEFLISSSQDGHQWTLFFQNGKVKVFQGNQDSFTPVVNSLDPPLLTRYLRIHPQSWVHQIALRMEVLGCEAQDLY Storage and Stability: Store product at 4C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20C. Aliquots are stable at -20C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Note: Applications are based on unconjugated antibody.
