The life cycle of coagulation factor VIII in view of its structure and function

PJ Lenting, JA Van Mourik… - Blood, The Journal of the …, 1998 - ashpublications.org
THE PAST TWO DECADES have brought remarkable progress in our understanding of the
molecular basis of hemophilia A. This disease, which has already been documented as a …

Function of von Willebrand factor in haemostasis and thrombosis

AJ Reininger - Haemophilia, 2008 - Wiley Online Library
The physiological protection against bleeding is secured by platelet adhesion to the site of
injury and sealing of the defect. The first step involves the arrest of platelets that have …

Structure of the C2 domain of human factor VIII at 1.5 Å resolution

KP Pratt, BW Shen, K Takeshima, EW Davie… - Nature, 1999 - nature.com
Human factor VIII is a plasma glycoprotein that has a critical role in blood coagulation,.
Factor VIII circulates as a complex with von Willebrand factor. After cleavage by thrombin …

Factor VIII and von Willebrand factor interaction: biological, clinical and therapeutic importance

V Terraube, JS O'donnell, PV Jenkins - Haemophilia, 2010 - Wiley Online Library
The interaction of factor VIII (FVIII) with von Willebrand Factor (VWF) is of direct clinical
significance in the diagnosis and treatment of patients with haemophilia A and von …

Role of the low density lipoprotein-related protein receptor in mediation of factor VIII catabolism

EL Saenko, AV Yakhyaev, I Mikhailenko… - Journal of Biological …, 1999 - ASBMB
In the present study, we found that catabolism of coagulation factor VIII (fVIII) is mediated by
the low density lipoprotein receptor-related protein (LPR), a liver multiligand endocytic …

Unwinding the von Willebrand factor strings puzzle

K De Ceunynck, SF De Meyer… - Blood, The Journal of …, 2013 - ashpublications.org
Abstract von Willebrand factor (VWF) is amongst others synthesized by endothelial cells and
stored as ultra-large (UL) VWF multimers in Weibel-Palade bodies. Although UL-VWF is …

Early increase of von Willebrand factor predicts adverse outcome in unstable coronary artery disease: beneficial effects of enoxaparin

G Montalescot, F Philippe, A Ankri, E Vicaut, E Bearez… - Circulation, 1998 - Am Heart Assoc
Background—The pathogenesis of unstable angina and non–Q-wave myocardial infarction
is still poorly understood, and early evaluation of prognosis remains difficult. We therefore …

The acidic region of the factor VIII light chain and the C2 domain together form the high affinity binding site for von willebrand factor

EL Saenko, D Scandella - Journal of Biological Chemistry, 1997 - ASBMB
A binding site for von Willebrand factor (vWf) was previously localized to the carboxyl
terminus of the C2 domain of the light chain (LCh) of factor VIII (fVIII). The acidic region of the …

[HTML][HTML] Activated protein C-catalyzed inactivation of human factor VIII and factor VIIIa. Identification of cleavage sites and correlation of proteolysis with cofactor activity …

PJ Fay, TM Smudzin, FJ Walker - Journal of Biological Chemistry, 1991 - Elsevier
Human factor VIII and factor VIIIa were proteolytically inactivated by activated protein C.
Cleavages occurred within the heavy chain (contiguous A1-A2-B domains) of factor VIII and …

von Willebrand factor: structure and function

D Meyer, JP Girma - Thrombosis and Haemostasis, 1993 - thieme-connect.com
Von Willebrand factor (vWF) pIays a key role in platelel adhesion, thrombus formation and
coagulation, the latter as carrier protein for factor VIII (F VIII). Its abnormalities lead to von …