MFRP is a molecular hub that organizes the apical membrane of RPE cells by engaging in interactions with specific proteins and lipids

Membrane frizzled-related protein (MFRP), present in the retinal pigment epithelium (RPE), is an integral membrane protein essential for ocular development and the normal physiology of the retina. Mutations in MFRP are associated with autosomal recessive nonsyndromic nanophthalmos, leading to severe hyperopia and early-onset retinitis pigmentosa. While several preclinical gene-augmentation and gene-editing trials hold promise for future therapies aimed at stopping degeneration and restoring retinal function, the molecular mechanisms involved in MFRP biology are still not well understood. Here, we studied the biochemical properties of MFRP and the molecular consequences of its loss of function in the retinal degeneration 6 (rd6) mouse model. Using transcriptomic and lipidomic approaches, we observed that accumulation of docosahexaenoic acid (DHA) constitutes a primary defect in the MFRP-deficient RPE. In biochemical assays, we showed that MFRP undergoes extensive glycosylation, and it pr..., , # Data from: MFRP is a molecular hub that organizes the apical membrane of RPE cells by engaging in interactions with specific proteins and lipids Dataset DOI: [10.5061/dryad.3r2280gxj](https://doi.org/10.5061/dryad.3r2280gxj) ## Description of the data and file structure We studied the biochemical properties of MFRP and the molecular consequences of its loss of function in the retinal degeneration 6 (rd6) mouse model by characterizing aspects of mouse biology and using transcriptomic, lipidomic and proteomic approaches. The dataset contains numerical data for plots within the manuscript. ### Files and variables #### File: Fig_3A_11-cis-retinal.txt **Description:** *Analysis of the contents of 11-cis-retinal in the eyes of fully dark-adapted rd6 and WT mice of different ages: 1-, 2-, 3-, and 6-mo. Values represent pmol/eye.* ##### Variables * age [months]: age of animal * MFRP (rd6) / wt: genotype of animal #### File: Fig_3A_Retinyl_esters.txt **Description:** *Analysis of the..., ,