Shh signaling directs murine kidney development by controlling a hierarchy of regulators, such as Gli family members (Gill and Rosenblum 2006), and inhibition of Hh signaling suppresses microcyst formation in human Autosomal Dominant Polycystic Kidney Disease cells and in mouse models (Silva et al. 2018; Tran et al. 2014). Here, GLI1 is linked to autosomal dominant polycystic kidney disease.