In addition, the activity of the VWF cleaving enzyme ADAMTS13 was measured in the Vwf+/+ mice, but did not change significantly compared to baseline samples (113.5 ± 18.2%, p = 0.053; Supplemental Fig. S2C) and were also not correlated with AAA severity (p = 0.962; Supplemental Fig. S2D). Here, ADAMTS13 is linked to triple-A syndrome.