Plays a role in the establishment of cell polarity and epithelial lumen formation (By similarity). Also plays an essential role in ciliogenesis and subsequent Hedgehog signaling pathway that requires the presence of intact primary cilia for pathway activation. Mechanistically, interacts with and mediates RABEP2 centrosomal localization which is critical for ciliogenesis (PubMed:27224062). Homodimer (By similarity). Interacts with OFD1; the interaction is direct (PubMed:20835237). Interacts with FAM161A (PubMed:22940612). Interacts with RABEP2, ERC1 and CEP131 (PubMed:27224062). Located at the distal ends of both centrioles and colocalizes to centrosomes throughout the cell cycle. Expressed in thymus, prostate, testis, ovary, small intestine, colon, mucosa, colon and renal cancer tumors. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry.