Glis2 has been proposed as a regulator of Hedgehog signaling required for maintaining kidney tubular cells in a differentiated state51; however, we have previously shown that the Hedgehog pathway has no role in ADPKD cyst progression in orthologous models71 so it is unlikely that this proposed role for Glis2 is related to its effector function in polycystin dependent signaling. This evidence concerns the gene GLIS2 and autosomal dominant polycystic kidney disease.