In relation to this, it is also worth noting that GPNMB (glycoprotein nonmetastatic B), a TFE3/TFEB target gene, is significantly upregulated in TSC/mTOR-mutated renal tumors (such as ESC RCC, LOT, AML, and PEComa), and that the levels are comparable to those found in translocation RCC, a tumor type driven by constitutive TFE3 (or TFEB) activation [106]. Here, TSC1 is linked to kidney neoplasm.