Target intelligence / Profile preview

Coagulation Factor VIII stabilization site on plasma proteins (FVIII stabilization site)

Target
FVIII stabilization site
Molecular classification
Protein-protein interaction, Coagulation factor, Plasma protein complex
01

Overview

The stabilization site for coagulation factor VIII (FVIII) on plasma proteins refers to the molecular interface and mechanisms by which FVIII is maintained in a stable, functional form within the bloodstream. The primary plasma protein responsible for this stabilization is von Willebrand factor (VWF), which binds FVIII and protects it from premature degradation, thereby ensuring its availability for hemostasis. This interaction is crucial for preventing bleeding disorders such as hemophilia A and von Willebrand disease. The C1 domain of FVIII is the primary binding site for the VWF D'D3 domain, with secondary contributions from the C2 domain and other regions. Disruptions in this interaction lead to FVIII instability and reduced clotting ability.

Other names
Factor VIII-VWF complexFVIII-VWF interaction siteVon Willebrand factor - Factor VIII binding site
02

Mechanism of action

Stabilization of FVIII through non-covalent binding to VWF, preventing degradation and ensuring availability for coagulation.

03

Biological functions

HemostasisBlood coagulationFactor VIII stabilizationVWF binding
04

Disease associations

Hemophilia AVon Willebrand diseaseThrombosis (indirectly)
05

Safety considerations

Development of FVIII inhibitors (antibodies)Thrombotic complications with VWF concentratesAnaphylaxis
06

Interacting drugs

Recombinant FVIII

2 more in the full profile.

07

Biomarkers

FVIII activityVWF antigen levelsFVIII inhibitor titers

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