POC1B is one of the two POC1 homologs that function together as a highly conserved core centriole and basal body component in vertebrates,15,32–34 invertebrates,35,36 and even Chlamydomonas reinhardtii32 and Tetrahymena thermophila.33 The other POC1 homolog, encoded by POC1A (previously Pix2 [MIM 614783]), shows protein structure and intracellular localization similar to those of POC1B.20,36 Studies in Tetrahymena thermophila suggest that POC1 proteins are essential for both structure and stability of the basal body.15 Depletion studies show that POC1B, unlike POC1A, is necessary for ciliogenesis, and typical ciliopathy-associated developmental defects (e.g., curved body axis, kidney cysts, and laterality defects) were described in poc1b morphant zebrafish. Interestingly, they were also reported to exhibit smaller eyes, but a more detailed ophthalmological analysis was not undertaken.15