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. Author manuscript; available in PMC: 2014 Sep 1.
Published in final edited form as: J Immunol. 2013 Jul 22;191(5):2184–2193. doi: 10.4049/jimmunol.1301221

FIGURE 3. ADAMTS13 deficiency leads to VWF-platelet string formation and C3 deposition under flow.

FIGURE 3

Histamine-pretreated PGEC cells were perfused with PRP. (A) Perfusion of PRP from TTP Patient 4 led to formation of VWF-platelet strings (arrowheads). The inset shows the VWF-platelet strings at a higher magnification. (B) rADAMTS13 was perfused over these strings (at the same location) at the same shear, clearing the strings within 3 mins. The inset at higher magnification demonstrates lack of strings. (C) VWF-platelet strings from TTP patients stained positively for C3 as shown here in a sample from Patient 3. Insets show C3 staining on the VWF-platelet strings from Patient 3 (left, higher magnification) and Patient 2 (right). (D) No C3 staining was visible on cells exposed to rADAMTS13 under flow. (E) Cells perfused with normal PRP pre-incubated with EDTA which inhibited the cleavage of VWF-platelet strings (arrowheads). (F) The strings shown in panel E did not stain for C3. Images are representative of six different experiments with reproducible results from five patients. Images were taken at 200x magnification (A-F and right inset in panel C), the left inset in panel C was taken at 400x magnification.