We also confirmed that tRCC pathologies, such as dilated renal tubules and development of renal carcinoma, seen in Cdh16‐Cre; Rosa26LSL‐PRCC‐TFE3/+ (tRCC) mice are suppressed in Cdh16‐Cre; RosaLSL‐PRCC‐TFE3/+; Angptl2Flox/Flox (CKO tRCC) mice in which ANGPTL2 expression is specifically deleted in renal tubular epithelial cells [13] (Fig. 1A). Here, ANGPTL2 is linked to renal cell carcinoma associated with Xp11.2 translocations/TFE3 gene fusions.