This mechanism has been proposed to be cilia-related simply because proteins involved in Bardet-Biedl syndrome (BBS), NPHP, orofacial digital syndrome type1 (OFD1), autosomal recessive polycystic kidney disease (ARPKD), and ADPKD localize to the cilium [94]. The gene discussed is OFD1; the disease is autosomal recessive polycystic kidney disease.