A similar ultrastructural phenotype of misaligned OS disks combined with mislocalized OS proteins and progressive photoreceptor cell loss has been observed in several mouse retinal dystrophy models bearing mutations in genes involved in OS development and maintenance, including Cngb1-Garp1-Garp2 (Zhang et al., 2009), Kif3a (Avasthi et al., 2009), Prom1 (Zacchigna et al., 2009; Dellett et al., 2014), and Rp1 (Gao et al., 2002; Liu et al., 2003; Song et al., 2014). Here, PROM1 is linked to inherited retinal dystrophy.