Decreased factor VIII levels during acetaminophen-induced murine fulminant hepatic failure

Activation of human endothelial cells from specific vascular beds induces the release of a FVIII storage pool

“…However, such a comparison must be taken with caution because there are clear differences in FVIII regulation between mice and humans. 11 The frequent use of HUVECs as source of human ECs and the heterogeneity in FVIII production between different EC types probably explain why, with the exception of liver sinusoidal ECs, the ECs were not recognized earlier as a source of FVIII. Note, Rosenberg et al 26 have analyzed FVIII secretion by HPMECs but have not detected measurable FVIII.…”

“…5 However, the study of the regulation of extrahepatic FVIII production has been hampered by the lack of identification of human extrahepatic tissues able to produce functional FVIII [6][7][8][9][10] and by the important difference in the regulation of plasma FVIII levels between humans and mice. 11 Recent observations have shown that human lungs secrete FVIII. 12 A significant production of FVIII has also been reported after transplantation of lungs from healthy dogs into hemophilic recipients.…”

High-level expression of porcine factor VIII from genetically modified bone marrow–derived stem cells

“…One-stage coagulation and chromogenic fVIII activity assays were performed as described previously. 8,15 A standard curve was generated by reconstituting plasma derived from hemophilia A mice with recombinant porcine fVIII. Production, purification, and characterization of recombinant BDD porcine fVIII has been described.…”

Hematopoietic stem-cell gene therapy of hemophilia A incorporating a porcine factor VIII transgene and nonmyeloablative conditioning regimens

“…10 Total anti-fVIII IgG levels were determined by enzyme-linked immunosorbent assay (ELISA) as described previously. 11,12 FVIII inhibitory activity was determined using a modified Bethesda assay 13 in which experimental plasma samples were incubated with human fVIII-deficient plasma reconstituted with recombinant BDD porcine fVIII for 2 hours and assayed for residual fVIII activity. 14 Intracellular staining for flow cytometry was performed using the Cytofix/Cytoperm kit according to the manufacturer's suggestions (BD Biosciences) with a biotinylated mouse anti-BDDpfVIII primary antibody.…”