ADGRV1 and retinal ciliopathy: Moreover, due to the presence of defects in photoreceptor cilia in cell and animal models for USH, evidence has been accumulating in recent years that USH is considered a retinal ciliopathy (Bujakowska et al., 2017; May-Simera et al., 2017; Grotz et al., 2022) which is consistent with the ciliary association of CIB2 and ADGRV1 described here.