KIZ (kizuna centrosomal protein) is a centrosomal protein essential for establishing robust mitotic centrosome architecture capable of withstanding forces generated during spindle formation 1. The protein localizes to the basal body of primary cilia in fibroblasts and the base of the photoreceptor connecting cilium in retinal tissue 1. KIZ functions by stabilizing expanded pericentriolar material around the centriole, supporting centrosome organization and spindle formation through protein-protein interactions 1. Mutations in KIZ cause retinitis pigmentosa 69 (RP69), a rod-cone dystrophy characterized by progressive photoreceptor degeneration 1. Multiple pathogenic variants have been identified, including nonsense mutations (c.226C>T, p.Arg76*; c.52G>T, p.Glu18*; c.3G>A, p.Met1?) and frameshift deletions (c.119_122del) 1. The disease mechanism appears complex: while mutations are predicted to trigger nonsense-mediated mRNA decay, patient-derived fibroblasts show unexpectedly preserved KIZ localization and normal cilia length, suggesting that retinal-specific pathogenic mechanisms may not be fully captured in fibroblast models 1. These findings indicate KIZ is critical for photoreceptor function and highlight the need for retinal-specific models, such as induced pluripotent stem cells, to elucidate the pathogenic mechanisms underlying KIZ-associated retinitis pigmentosa.