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A constant feature is early-onset retinal degeneration leading to blindness. Among the most common forms is BBS type 10 (BBS10), which is caused by mutations in a gene encoding a chaperonin-like protein. To aid in developing treatments, we phenotyped a Bbs10 knockout (Bbs10\u2212\/\u2212) mouse model. Analysis by optical coherence tomography (OCT), electroretinography (ERG) and a visually guided swim assay (VGSA) revealed a progressive degeneration (from P19 to 8\u00a0months of age) of the outer nuclear layer that is visible by OCT and histology. Cone ERG was absent from at least P30, at which time rod ERG was reduced to 74.4% of control levels; at 8\u00a0months, rod ERG was 2.3% of that of controls. VGSA demonstrated loss of functional vision at 9\u00a0months. These phenotypes progressed more rapidly than retinal degeneration in the Bbs1M390R\/M390R knock-in mouse. This study defines endpoints for preclinical trials that can be utilized to detect a treatment effect in the Bbs10\u2212\/\u2212 mouse and extrapolated to human clinical trials.<\/jats:p>","DOI":"10.1242\/dmm.049473","type":"journal-article","created":{"date-parts":[[2022,9,20]],"date-time":"2022-09-20T03:39:14Z","timestamp":1663645154000},"source":"Crossref","is-referenced-by-count":12,"title":["Progressive retinal degeneration of rods and cones in a Bardet-Biedl syndrome type 10 mouse model"],"prefix":"10.1242","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6605-5846","authenticated-orcid":false,"given":"Sara K.","family":"Mayer","sequence":"first","affiliation":[{"name":"Interdisciplinary Graduate Program in Genetics, University of Iowa 1 , Iowa City, IA 52242 , USA"},{"name":"Institute for Vision Research, University of Iowa 2 Department of Ophthalmology and Visual Sciences , , Iowa City, IA 52242 , 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