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 3
Plasma vWF-cleaving activity and vWF multimer size distribution for ADAMTS13-deficient mice and littermate controls. (A) vWF-cleaving activity in plasma from Adamts13^B/129+/+, Adamts13^B/129+/–, and Adamts13^B/129–/– mice measured using purified human full-length vWF as a substrate. Activity was determined by analysis of the specific vWF-cleavage product indicated by the arrow, and comparison to the pattern in the control lane with ADAMTS13 activity completed inhibited by EDTA, as indicated. Specific ADAMTS13 activity was seen in plasma from Adamts13^B/129+/+ and Adamts13^B/129+/– mice but not Adamts13^B/129–/– mice. (B) vWF-cleaving activity in plasma from Adamts13^B/129+/+, Adamts13^B/129+/–, and Adamts13^B/129–/– mice determined using recombinant murine GST/vWF A2 domain as a substrate, which contains the previously identified ADAMTS13 cleavage site. Activity was demonstrated by appearance of the expected cleavage product indicated by the arrow. Specific ADAMTS13 activity was seen in plasma from Adamts13^B/129+/+ and Adamts13^B/129+/– mice but not Adamts13^B/129–/– mice. Serial dilution of Adamts13^B/129+/+ plasma demonstrated sensitivity to less than 1.5% of control plasma activity. (C) The vWF multimer distribution in plasma from Adamts13^B/129+/+ and Adamts13^B/129–/– mice. (D) The vWF multimer distribution in plasma from Adamts13^B/CN2+/+ and Adamts13^B/CN2–/– mice. The approximate range of UL-vWF is indicated by the brackets.