BOEC characterization: type 1 VWD. Data from patient VWD-1 (A) and patient VWD-12 (B). (Ai,Bi) VWF was measured by ELISA in the total cell lysate (left) and cell culture supernatant (right) from BOECs isolated from the indicated patient in the absence and presence of PMA stimulation (mean ± SEM from 3 replicates). (Aii,Bii) VWF expression from control or PMA-stimulated BOECs was visualized by confocal IF microscopy (scale bars represent 20 µm). Boxes show 2× magnified image. EM analysis of BOECs show elongated (Aiii,Biii) or rounded WPBs (Biii) (scale bars represent 200 nm in Aiii and 500 nm in Biii). (Aiv) Mature and propeptide-containing VWF was detected by western blotting from a healthy control and VWD-1. Equal amounts of VWF were loaded per lane.