Figure 6.

BOEC characterization: type 2M VWD family. Data from patient VWD-2 (A) and patient VWD-3 (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 in control or PMA-stimulated BOECs was visualized by confocal IF microscopy (scale bars represent 20 µm). Boxes show 2× magnified image. (Biii) Representative image from EM analysis (scale bar represents 500 nm) of BOECs from patient VWD-3 illustrates the presence of morphologically normal WPBs. *P < .05; ** P < .01; ***P < .001.