CSN1S1 and thrombotic thrombocytopenic purpura: ADAMTS13-deficient mice (with the 129X1/SvJ and C57BL/6J genetic background) did not develop TTP-like pathology spontaneously but, upon introduction of the CASA/Rk background, were shown to develop TTP-like pathology after endothelial cell injury was induced by Shiga toxin [21].