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RP12 results in progressively worsening peripheral vision, whereas LCA8 causes severe visual impairment at birth or in early life. While several mouse models have been proposed for RP12, few replicate the full spectrum of human LCA8 pathology, such as disorganized retinal layering, abnormal retinal thickening, pigmentary defects, hyperreflective lesions, and severely attenuated electroretinogram responses at birth. Six models have been proposed utilizing the Cre\u2010loxP system to delete candidate genes in specific retinal cell types and developmental stages. The model ablating <jats:italic>Crb1<\/jats:italic> and its homolog <jats:italic>Crb2<\/jats:italic> (using mRx\u2010Cre) from the beginning of the eye development is the most complete as it shows blindness during the eye\u2010opening stage, pigmentary defects in the RPE, ganglion cell layer heterotopia, disruption of retinal lamination, and acellular patches. LCA8 represents a unique type of retinal dystrophy among LCA subtypes, driven by dysfunctional retinal progenitor cells during eye development. In contrast, other LCA types and RP12 are caused by photoreceptor defects. Therefore, the most accurate LCA8\u2010like mouse model must target both alleles of the <jats:italic>Crb1<\/jats:italic> and <jats:italic>Crb2<\/jats:italic> genes in the optic vesicle or earlier.<\/jats:p>","DOI":"10.1002\/dvdy.462","type":"journal-article","created":{"date-parts":[[2022,2,11]],"date-time":"2022-02-11T22:08:35Z","timestamp":1644617315000},"page":"1094-1106","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Current perspectives in Leber congenital amaurosis type 8 mouse modeling"],"prefix":"10.1002","volume":"251","author":[{"given":"Ankur","family":"Nahar","sequence":"first","affiliation":[{"name":"Thomas Jefferson University Sidney Kimmel Medical College  Philadelphia Pennsylvania 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