The autosomal dominant Retinitis Pigmentosa (RP) is a neurodegenerative disease commonly caused by the misfolding opsins that aggregate in the endoplasmic reticulum (ER) during the mutation of Proline-to-Histidine substitution in cordon 23 (P23H).
The aggregation due to the increase of beta-sheet deregulates the chemical composition of retinal layers. To provide a better understanding and therapeutic issues of the disease, Fourier Transform Infrared Spectroscopy Imaging (FTIR) was used to study and quantify the chemical composition of mice retinal layers (wild type and mutant samples).
The results obtained from statistical data analysis by comparing both groups (wild type and mutant samples) suggest that the misfolding opsins (P23H) in the photoreceptor layer disrupt the chemical composition (both protein and lipid composition) of retinal layers especially the outer nuclear and the outer plexiform layers.
In fact, by comparing the wild-type and mutant samples, we found that the lipid composition is significantly deregulated in mutant samples. This deregulation affects the transmission process in the retina.