DIS3L2 and Perlman syndrome: While LIF/2i-adapted DIS3L2-deficient ESCs remain undifferentiated, the greater competence of these cells to respond to differential stimuli after an in vitro, and perhaps in vivo, exit from pluripotency results in enhanced renal lineage differentiation program and eventually leads to kidney overgrowth, similar to that in the Perlman syndrome patients.