Accumulating studies have demonstrated an activated Shh signaling in fibrotic CKD, suggesting a potential connection between aberrant regulation of this signaling and kidney fibrosis (Ding et al., 2012; Fabian et al., 2012; Kramann et al., 2015; Rauhauser et al., 2015; Zhou et al., 2014). Here, SHH is linked to chronic kidney disease.