Figure 3
Normal and patient BOEC VWF immunofluorescent confocal images. (A) Normal BOECs with rod-shaped WPBs. (B) Patient V70 (c.3538G>A) with punctate VWF as well as diffuse VWF staining throughout the cells. (C) Type 3 patient T151 (c.3939G>A, c.5842+1G>C) with few punctate WPBs. (D) Patient T152 (c.5842+1G>C) BOECs show not only more classic, but also rounded WPBs. (E) Patient V447’s (c.6599-20A>T) VWF appears diffuse within the cell as well as localized to punctate WPBs. (F) VWF staining in patient V449’s (c.6599-20A>T) appear relatively normal with many rod-shaped WPBs.). Imaging was conducted at room temperature using a Quorum Wave Effects Spinning Disc confocal microscope at ×60 magnification and captured with a Hamamatsu Orca high-resolution camera and ImageJ software analysis. Scale bar, 5 μM.

Normal and patient BOEC VWF immunofluorescent confocal images. (A) Normal BOECs with rod-shaped WPBs. (B) Patient V70 (c.3538G>A) with punctate VWF as well as diffuse VWF staining throughout the cells. (C) Type 3 patient T151 (c.3939G>A, c.5842+1G>C) with few punctate WPBs. (D) Patient T152 (c.5842+1G>C) BOECs show not only more classic, but also rounded WPBs. (E) Patient V447’s (c.6599-20A>T) VWF appears diffuse within the cell as well as localized to punctate WPBs. (F) VWF staining in patient V449’s (c.6599-20A>T) appear relatively normal with many rod-shaped WPBs.). Imaging was conducted at room temperature using a Quorum Wave Effects Spinning Disc confocal microscope at ×60 magnification and captured with a Hamamatsu Orca high-resolution camera and ImageJ software analysis. Scale bar, 5 μM.

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