Figure 2.

Mice lacking the Stx2-receptor Gb3 in tubular epithelium developed cerebral purpura after Stx2-injection. (A) Although Pax8^cre/Ugcg^fl/fl mice were protected from kidney failure and electrolyte dysregulation (shown in Figure 1F), severe and partially fatal neurological symptoms occurred 4–8 days after the Stx2 injection. (B, C) Autopsy revealed diffuse cerebral purpura (B) with bleeding and cerebral TMA; Goldner's trichrome staining, visualizing red blood cells in the haemorrhage foci; magnification = ×200. (D) Depletion of Gb3 in endothelial cells in addition to the tubular cells (Pax8^cre/Tie2^cre/Ugcg^fl/fl, green line) conferred a partial protection from cerebral purpura and improved survival significantly as compared to pure tubular depletion of Gb3 (Pax8^cre/Ugcg^fl/fl, blue line). In contrast, depletion of Gb3 in platelets in addition to tubular cells (Pax8^cre/PF4^cre/Ugcg^fl/fl, yellow line) did not prove to be protective. As expected, mice with Gb3 deficiency solely in platelets or endothelial cells (PF4^cre/Ugcg^fl/fl and Tie2^cre/Ugcg^fl/fl, respectively) died during the early phase, contemporarily with the Stx2-exposed WT mice.