This is important because hyperglutamylation induced by CCP5 depletion, can effectively restore axonemal glutamylation, the ciliary dosage of polycystins, and Shh signaling in human wild-type or Autosomal dominant polycystic kidney disease (ADPKD) cells (He et al., 2018), which highlights the perspective that augmenting ciliary signaling by increasing axoneme glutamylation may represent an intriguing therapeutic strategy for certain ciliopathies. Here, AGBL5 is linked to ciliopathy.