Importantly, autosomal dominant polycystic kidney disease (ADPKD) protein polycystins and Sonic Hedgehog (Shh) signaling molecule GLI3 lost their specific cilia targeting in glutamylation-deficient cilia, suggesting an unexpected role for axoneme polyglutamylation in anchoring/trafficking of signaling receptor/molecules in the context of cilia. This evidence concerns the gene SHH and autosomal dominant polycystic kidney disease.