ASPSCR1 and Renal insufficiency: Similar to previous studies with constitutive expression of ASPSCR1-TFE3 26, wild-type TFEB55, or inactivation of FLCN 56,57 during renal development, expression of SFPQ-TFE3 using Ksp-Cadherin-Cre (SFPQ-TFE3LSL; Ksp-Cre mice; STK) resulted in disrupted renal development and renal insufficiency, culminating in early neonatal death.