To evaluate the effect of AL101 in vivo, we selected two ACC PDX models reported to harbor NOTCH1 activating alterations: ACCx9, which carries an activating I1680N mutation; and ACCx11, which harbors a tandem duplication 3′ of NOTCH1 and has high levels of NICD1 [15]. This evidence concerns the gene NOTCH1 and adrenal cortex carcinoma.