[HTML][HTML] Dangerous liaisons: how the immune system deals with factor VIII

A Wroblewska, BM Reipert, KP Pratt… - Journal of Thrombosis and …, 2013 - Elsevier
Only a fraction of patients with hemophilia A develop a neutralizing antibody (inhibitor)
response to therapeutic infusions of factor VIII. Our present understanding of the underlying …

To serve and protect: the modulatory role of von Willebrand factor on factor VIII immunogenicity

RB Hartholt, AS van Velzen, I Peyron, A Ten Brinke… - Blood reviews, 2017 - Elsevier
Hemophilia A is a bleeding disorder characterized by the absence or dysfunction of blood
coagulation factor VIII (FVIII). Patients are treated with regular infusions of FVIII concentrate …

Molecular determinants of the factor VIII/von Willebrand factor complex revealed by BIVV001 cryo-electron microscopy

JR Fuller, KE Knockenhauer, NC Leksa… - Blood, The Journal …, 2021 - ashpublications.org
Interaction of factor VIII (FVIII) with von Willebrand factor (VWF) is mediated by the VWF D′
D3 domains and thrombin-mediated release is essential for hemostasis after vascular injury …

[HTML][HTML] Shear stress is required for the endocytic uptake of the factor VIII‐von Willebrand factor complex by macrophages

L Castro‐Nunez, I Dienava‐Verdoold… - Journal of Thrombosis …, 2012 - Elsevier
Background: Low‐density lipoprotein (LDL) receptor family members contribute to the
cellular uptake of factor VIII. How von Willebrand factor fits into this endocytic pathway has …

Structural basis for inhibition of coagulation factor VIII reveals a shared antigenic hotspot on the C1 domain

KC Childers, B Cowper, JD Vaughan, JR McGill… - Journal of Thrombosis …, 2024 - Elsevier
Background Hemophilia A arises from dysfunctional or deficient coagulation factor (F) VIII
and leads to inefficient fibrin clot formation and uncontrolled bleeding events. The …

[HTML][HTML] The C1 and C2 domains of blood coagulation factor VIII mediate its endocytosis by dendritic cells

B Gangadharan, M Ing, S Delignat, I Peyron… - …, 2017 - ncbi.nlm.nih.gov
The development of inhibitory antibodies to therapeutic factor VIII is the major complication
of replacement therapy in patients with hemophilia A. The first step in the initiation of the anti …

A major determinant of the immunogenicity of factor VIII in a murine model is independent of its procoagulant function

SL Meeks, CL Cox, JF Healey… - Blood, The Journal …, 2012 - ashpublications.org
A main complication of treatment of patients with hemophilia A is the development of anti–
factor VIII (fVIII) antibodies. The immunogenicity of fVIII potentially is a function of its …

Engineered tissue inhibitor of metalloproteinases-3 variants resistant to endocytosis have prolonged chondroprotective activity

CM Doherty, R Visse, D Dinakarpandian… - Journal of Biological …, 2016 - ASBMB
Tissue inhibitor of metalloproteinases-3 (TIMP-3) is a central inhibitor of matrix-degrading
and sheddase families of metalloproteinases. Extracellular levels of the inhibitor are …

Factor VIII Interacts with the Endocytic Receptor Low-density Lipoprotein Receptor-related Protein 1 via an Extended Surface Comprising “Hot-Spot” Lysine Residues♦

M Van Den Biggelaar, JJ Madsen, JH Faber… - Journal of Biological …, 2015 - ASBMB
Lysine residues are implicated in driving the ligand binding to the LDL receptor family.
However, it has remained unclear how specificity is regulated. Using coagulation factor VIII …

Modification of an exposed loop in the C1 domain reduces immune responses to factor VIII in hemophilia A mice

A Wroblewska, SD van Haren… - Blood, The Journal …, 2012 - ashpublications.org
Abstract Development of neutralizing Abs to blood coagulation factor VIII (FVIII) provides a
major complication in hemophilia care. In this study we explored whether modulation of the …