Structure and Function of Recombinant versus Plasma-Derived von Willebrand Factor and Impact on Multimer Pharmacokinetics in von Willebrand Disease.

ABSTRACT:

Background

Recombinant von Willebrand factor (rVWF, vonicog alfa) is a purified VWF concentrate produced from Chinese hamster ovary cells. rVWF is not exposed to the VWF-cleaving protease ADAMTS13 and so is not subject to proteolytic degradation of large (L) and ultra-large (UL) VWF multimers by that enzyme.

Purpose

To compare the structure and function of rVWF with the human plasma-derived VWF [pdVWF] concentrates Haemate P^®/Humate-P^®, Voncento^®, Wilate^®/Eqwilate^®, and Wilfactin^®/Willfact^®; to investigate the relationship between VWF multimeric pattern and VWF:ristocetin cofactor (VWF:RCo) activity through population pharmacokinetic (PK) modeling in patients with severe von Willebrand disease (VWD)

SUBMITTER: Gritsch H 

PROVIDER: S-EPMC9673800 | biostudies-literature | 2022

REPOSITORIES: biostudies-literature