Alternative trafficking of Weibel-Palade body proteins in CRISPR/Cas9-engineered von Willebrand factor-deficient blood outgrowth endothelial cells.
Maaike SchillemansMarije KatJurjen WestenengAnastasia GangaevMenno HofmanBenjamin NotaFloris P J van AlphenMartin de BoerMaartje van den BiggelaarCoert MargadantJan VoorbergRuben BieringsPublished in: Research and practice in thrombosis and haemostasis (2019)
CRISPR editing of VWF provides a robust method to create VWF- deficient BOECs that can be directly compared to their wild-type counterparts. Results obtained with our model system confirmed alternative trafficking of several WPB proteins in the absence of VWF and support the theory that increased Ang-2/Tie-2 interaction contributes to angiogenic abnormalities in VWD patients.