Component of the centrioles controlling mother and daughter centrioles length. Recruits to the centriole IFT88 and centriole distal appendage-specific proteins including CEP164. Involved in the biogenesis of the cilium, a centriole-associated function. The cilium is a cell surface projection found in many vertebrate cells required to transduce signals important for development and tissue homeostasis. Plays an important role in development by regulating Wnt signaling and the specification of the left-right axis. Only OFD1 localized at the centriolar satellites is removed by autophagy, which is an important step in the ciliogenesis regulation. Homooligomer. Interacts with LCA5. Interacts with RUVBL1; the interaction is direct and may mediate interaction with the NuA4 histone acetyltransferase complex. Interacts with SDCCAG8; the interaction is direct. Interacts with MAP1LC3B. Interacts with C2CD3; OFD1 may act as a negative regulator of C2CD3. Forms a complex with KIAA0753/OFIP and CEP20/FOR20; the interaction with CEP20 is detected only in the presence of KIAA0753. Interacts with PCM1; this interaction may be mediated by KIAA0753/OFIP (PubMed:26643951). Localizes to centriole distal ends and to centriolar satellites (PubMed:20230748, PubMed:24089205). Localization to centrioles and pericentriolar satellites may be mediated by KIAA0753/OFIP (By similarity). Females die at birth and display severe craniofacial and limb abnormalities associated with disorganization of the brain, reduction of the lungs, defects in the great vessels and cystic kidney. Primary cilia are absent on the luminal surface of glomerular and tubular cells of kidneys. Males die earlier during development of the embryo, display failure of left right axis specification associated with a lack of cilia in the embryonic node. Belongs to the OFD1 family.