Compensation of inner retina to early-stage photoreceptor degeneration in a Rho(P23H/+) mouse model of retinitis pigmentosa

在Rho(P23H/+)视网膜色素变性小鼠模型中,内层视网膜对早期感光细胞退化的代偿作用

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Abstract

Retinitis pigmentosa (RP) is an inherited retinal disorder characterized by the degeneration of photoreceptors. Rho(P23H/+) mice, which carry a Pro23His mutation in the RHODOPSIN (Rho) gene, are one of the most studied animal models for RP. However, except for the photoreceptors, other retinal neural cells have not been fully investigated in this model. Here, we record the temporal changes of the retina by optical coherence tomography (OCT) imaging of the Rho(P23H/+) mice, from early to mid-phase of retinal degeneration. Based on thickness analysis, we identified a natural retinal thickness adaption in wild-type mice during early adulthood and observed morphological compensation of the inner retina layer to photoreceptor degeneration in the Rho(P23H/+) mice, primarily on the inner nuclear layer (INL). Rho(P23H/+) mice findings were further validated via: histology showing the negative correlation of INL and ONL thicknesses; as well as electroretinogram (ERG) showing an increased b-wave to a-wave ratio. These results unravel the sequential morphologic events in this model and suggest a better understanding of retinal degeneration of RP for future studies.

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