Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice
J. Clin. Invest. David G. Motto, et al. 115:2752 doi:10.1172/JCI26007 [Go to this article.]

Figure 7
ADAMTS13 deficiency prolongs vWF-mediated platelet–endothelial cell interactions in vivo. (A) Fluorescently labeled platelets began to adhere to the endothelium of mesenteric venules within 30–45 seconds after superfusion of calcium ionophore A23187. The adhesion was short lived in +/+ mice, whereas it was longer lived in ADAMTS13-deficient mice. Arrowheads indicate attached strings of platelets at least 20 μm in length. The insets indicate time after superfusion of A23187. (B) Quantification of fluorescent platelets sticking and/or translocating on vWF in A23187-activated venules. Adhesion of platelets to the vessel wall reached a peak between 45 seconds and 1 minute following superfusion of A23187 and progressively decreased with time. This process was absent in Vwf^–/– mice. Significantly more platelet adhesion was observed after 2 minutes and continued longer in the Adamts13^B/129–/– and Adamts13^B/CN1–/– mice compared with littermate +/+ controls. In 3 of 8 Adamts13^B/129–/– mice, platelet adhesion continued through 16 minutes. Similarly, in 3 of 7 Adamts13^B/CN1–/– mice, platelet adhesion continued through 35 minutes. *P < 0.05. (C) Fluorescent platelets aligned with a “beads-on-a-string” appearance on vWF in activated mesenteric venules. Very long strings up to 180 μm were observed. Video recordings of these experiments are provided in the supplemental material.