Finally, variants in ANKS6 (Ankyrin Repeat and Sterile Alpha Motif Domain Containing 6), which encodes a protein that interacts with other proteins of the Inv compartment of cilium (NEK8, NPHP2/INVS, and NPHP3), were detected in other patients who had histological periportal fibrosis and cholestatic hepatopathy along with a picture of infantile nephronopthisis-related ciliopathy (NPHP-RC) [13, 14]. Here, NPHP3 is linked to Periportal fibrosis.