Correspondence to: Dr. Anna Pavlova Institut für Experimentelle Hämatologie und Transfusionsmedizin , Universitätsklinikum Bonn Sigmund-Freud-Str. 25, 53127 Bonn, Germany Tel. +49/(0)/228/28 71 97 11, Fax +49/(0)228/28 71 60 87 E-mail: [email protected] Hämostaseologie 2010; 30 (Suppl 1): S153–S155 Human blood coagulation factor VIII (FVIII) is an essential protein for the blood coag...

Membrane-binding of a human blood clotting protein, factor VIII (FVIII), was studied by cryoelectron microscopy. Well visible gap junctions between adjacent phosphatydyl serine containing phospholipid vesicles were observed upon FVIII association. Digital images acquired with a JEOL2010FEG transmission electron microscope equipped with a 4x4k CCD camera were analysed with the Digital Micrograph...

Molecular defects in Factor VIII (FVIII), such as haemophilia A-related mutations or denaturative conformational changes, may affect the stability of FVIII as well as its interactions with physiological activators, von Willebrand Factor, phospholipid, or conformationally sensitive antibodies. We summarize the contemporary assays which allow identification of impaired functional interactions of ...

dis or der af fect ing ap prox i mately 1 in 10,000 male. The dis ease is caused by a de fi ciency of co ag u la tion fac tor VIII (FVIII) re sult ing from mu ta tions in the FVIII gene. Nu cle o tide sub sti tu tions/in ser tions of var i ous sizes have been iden ti fied through out the FVIII gene. This ge netic di ver sity ac counts for the ob served het er o ge ne ity in clin i cal se ver it...

Introduction Plasma factor VIII (FVIII) and von Willebrand factor (VWF) levels have been associated with the rate and severity of arterial thrombus formation and have been linked to outcomes following thrombolytic therapy in acute myocardial infarction patients. Here, we aimed to investigate FVIII and VWF levels during the course of thrombolysis in acute ischemic stroke (AIS) patients and to fi...

The primary cellular source of FVIII biosynthesis is controversial, with contradictory evidence supporting an endothelial or hepatocyte origin. LMAN1 is a cargo receptor in the early secretory pathway that is responsible for the efficient secretion of FV and FVIII to the plasma. Lman1 mutations result in combined deficiency of FV and FVIII, with levels of both factors reduced to ~10-15% of norm...

The effect of replacement therapy has significantly improved the morbidity and mortality of people with haemophilia A in high income countries, a recent socio-economic development as the availability of safe concentrates has been matched by a willingness for their provision through reimbursement. In the developing world, however, this state has not been achieved, primarily because of the low vi...

Today it is generally accepted that B cells require cognate interactions with CD4(+) T cells to develop high-affinity antibodies against proteins. CD4(+) T cells recognize peptides (epitopes) presented by MHC class II molecules that are expressed on antigen-presenting cells. Structural features of both the MHC class II molecule and the peptide determine the specificity of CD4(+) T cells that ca...

Organ transplantation and perfusion studied indicate that the spleen plays an important role in the regulation of plasma levels of factor VIII-von Willebrand's factor (FVIII-vWF). To better understand the mechanisms that regulate the FVIII-vWF increases after infusion of 1-deamino-8-D-arginine vasopressin (DDAVP), we have measured factor VIII coagulant activity (FVIII:C) and antigen (FVIII:CAg)...

The B domain of the coagulation factor (F)VIII comprises some unique characteristics. Though the B domain is important for processing, intracellular transport and secretion of FVIII protein, its role in the coagulation still remains unclear. This study aims to investigate the influence of 19 reported B domain variants on quantity and quality of expressed FVIII protein. F8 variants were transien...