Retinal proteome profiling of inherited retinal degeneration across three different mouse models suggests common drug targets in retinitis pigmentosa

Inherited retinal degenerations (IRDs) are a leading cause of blindness among the population of young people in the developed world. Approximately half of IRDs initially manifest as a gradual loss of night vision and visual fields, characteristic of retinitis pigmentosa (RP). Due to challenges in genetic testing and the large heterogeneity of mutations underlying RP, targeted gene therapies are an impractical large-scale solution in the foreseeable future. For this reason, identifying key pathophysiological pathways in IRDs that could be targets for mutation-agnostic and disease-modifying therapies (DMTs) is warranted. In this study, we investigated the retinal proteome of three distinct IRD mouse models, in comparison to sex- and age-matched wild-type mice. Specifically, we used the Pde6βRd10 (rd10) and RhoP23H/WT (P23H) mouse models of autosomal recessive and autosomal dominant RP, respectively, as well as the Rpe65^-/-^ mouse model of Leber's congenital amaurosis type 2 (LC..., Animal Models and Study Design
We used three different mouse models of inherited retinal degenerative diseases (IRD) to discover retinal proteomic changes that are common to retinal degeneration (RD). The models used in this study were B6.CXB1-Pde6brd10/J (RRID: IMSR_JAX:004,297, referred to as rd10), B6.129S6(Cg)-Rhotm1.1Kpal/J (RRID: IMSR_JAX:017,628, referred to as P23H), mouse models of recessive and autosomal dominant retinitis pigmentosa (RP), respectively (18, 19), and B6.129-Rpe65tm1Tmr/J (RRID: IMSR_JAX:035,329, referred to as Rpe65−/−) model of Leber congenital amaurosis type 2 (LCA2) which was a kind gift from Dr Michael Redmond (National Institutes of Health) (20). The rd10 colony was kept as a homozygote. Age- and sex-matched C57BL/6J mice (RRID: IMSR_JAX:000,664) were used as controls. P23H heterozygote mice were bred with C57BL/6J mice, yielding P23H heterozygote and wild-type (WT) littermates. To get Rpe65−/− and their WT littermate mice, we bred hete..., # Retinal proteome profiling of inherited retinal degeneration across three different mouse models suggests common drug targets in retinitis pigmentosa

Dataset DOI: [10.5061/dryad.c2fqz61pc](10.5061/dryad.c2fqz61pc)

## Description of the data and file structure

### Files and variables

This dataset is showing all proteins identified in indicated group of samples, combined by genotype/group.

**Files (data generated at UCI, US):**

* protDDA_Rd10_DR.csv
* protDDA_Rd10_CLR.csv
* protDDA_P23H.csv
* protDDA_RPE65_KO.csv

**Variables:**
* P23H_het_M - heterozygous mice for P23H mutation in Rhodopsin, Male
* P23H_het_F - heterozygous mice for P23H mutation in Rhodopsin, Female
* Rd10_M - A mouse genotype with a point mutation in the Pde6b gene, leading to progressive photoreceptor (especially rod) degeneration, used as a model for retinitis pigmentosa, Male
* Rd10_F - A mouse genotype with a point mutation in the Pde6b gene, leading to progressive photoreceptor (especially rod) degenerat...,