Although limited USH2A-related patient-derived retinal organoids have been published, several retinal organoids modelling ciliopathy-related retinal diseases have successfully recapitulated key features of patient retinal phenotype, such as CEP290-LCA related ciliopathy (Parfitt et al., 2016), RP2-related RP (Schwarz et al., 2017) and RPGR-associated RP (Deng et al., 2018). This evidence concerns the gene CEP290 and retinitis pigmentosa 1.