Furthermore, the extreme discordance toward TSC2 mutation in the angiomyolipoma TSC patients enrolled in the EXIST-2 trial, 97% TSC2vs 3% TSC1 (P<0.0001), also fits the high odds ratio seen for angiomyolipomas in favor of TSC2 mutations in previous studies.33 Clinically, this finding translates to the simple clinical inference that TSC subjects with significant renal angiomyolipoma are much more likely to have a TSC2 mutation than a TSC1 mutation. Here, TSC1 is linked to tuberous sclerosis.