PURPOSE: To validate the Cdhr1-/- mouse as a model for human CDHR1-associated retinal degeneration, which may present as cone-rod dystrophy or geographic atrophy. METHODS: Deep phenotyping of Cdhr1-/-(n = 56) and C57BL6J wildtype control mice (n = 45) was undertaken using in vivo multimodal retinal imaging and dark- and light-adapted electroretinography (ERG) over 15 months to evaluate rod- and cone-photoreceptor responses and retinal morphology. RESULTS: Cdhr1-/- retinas exhibited outer retinal thinning on optical coherence tomography (OCT) at 1-month versus C57BL6J (mean 14.6% reduction; P
Journal article
Exp Eye Res
06/05/2021
208
CDHR1, Cadherin-related family member 1, Cone-rod dystrophy, Electroretinography, Light toxicity, Macular degeneration, Macular dystrophy, Optical coherence tomography, Retinitis pigmentosa