Fig. 4.
Fig. 4. Intracellular storage of dimeric VWF in granules. / Intracellular storage of dimeric VWF in granules is similar to that of wild-type VWF. Indirect immunofluorescence detection of VWF storage granules in stably transfected AtT-20 cells demonstrates the vesicular storage of dimeric VWF. AtT-20 cells were stably transfected with WT-VWF (panel A) or Tyr87Ser-VWF (panel B). The fixed and permeabilized cells were immunolabeled as described in “Materials and methods.” All cells shown in this Figure have been labeled with anti-VWF polyclonal antibodies (detected by Texas Red) to visualize the intracellular localization of VWF. Shown are 3-D projections of confocal images taken by confocal laser scanning microscopy (Bio-Rad C600, × 1500 magnification). Dimeric VWF resulting from the Tyr87Ser mutation is stored in Weibel-Palade body–like granules similar to those of WT-VWF storage.

Intracellular storage of dimeric VWF in granules.

Intracellular storage of dimeric VWF in granules is similar to that of wild-type VWF. Indirect immunofluorescence detection of VWF storage granules in stably transfected AtT-20 cells demonstrates the vesicular storage of dimeric VWF. AtT-20 cells were stably transfected with WT-VWF (panel A) or Tyr87Ser-VWF (panel B). The fixed and permeabilized cells were immunolabeled as described in “Materials and methods.” All cells shown in this Figure have been labeled with anti-VWF polyclonal antibodies (detected by Texas Red) to visualize the intracellular localization of VWF. Shown are 3-D projections of confocal images taken by confocal laser scanning microscopy (Bio-Rad C600, × 1500 magnification). Dimeric VWF resulting from the Tyr87Ser mutation is stored in Weibel-Palade body–like granules similar to those of WT-VWF storage.

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