Figure 3.

BOEC characterization: type 1 VWD. Data from patient VWD-10 (A) and patient VWD-8 (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. (Biii) Mature and propeptide-containing VWF was detected by western blotting from a healthy control or VWD patient VWD-8. Equal amounts of VWF were loaded per lane. (Biv) Representative image from EM analysis of BOECs from VWD patient VWD-8 shows extensive ER (scale bar represents 200 nm).