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Published on: March 20, 2021
Rod-derived cone viability factor for treating blinding diseases: from clinic to redox signaling
Thierry Léveillard1, José-Alain Sahel
1Department of Genetics, Institut de la Vision, INSERM, UPMC University of Paris 06, UMR-S 968, CNRS 7210, Paris F-75012, France. thierry.leveillard@inserm.fr
Abstract:
The identification of one mechanism that causes vision loss in inherited degenerative retinal disorders revealed a new signaling molecule that represents a potential therapy for these currently untreatable diseases. This protein, called rod-derived cone viability factor (RdCVF), maintains the function and consequently the viability of cone photoreceptor cells in the retina; mice that lack this factor exhibit a progressive loss of photoreceptor cells. The gene encoding RdCVF also encodes, by differential splicing, a second product that has characteristics of a thioredoxin-like enzyme and protects both photoreceptor cells and, more specifically, its interacting protein partner, the tau protein, against oxidative damage. This signaling pathway potentially links environmental insults to an endogenous neuroprotective response.
Insights
A newly discovered protein, rod-derived cone viability factor (RdCVF), offers potential therapy for inherited retinal diseases. This factor is crucial for maintaining cone photoreceptor cells, and its absence leads to vision loss.
Area of Science:
- Ophthalmology
- Neuroscience
- Molecular Biology
Background:
- Inherited degenerative retinal disorders cause progressive vision loss.
- Current treatments for these conditions are limited.
- A key mechanism underlying vision loss is being investigated.
Purpose of the Study:
- To identify novel therapeutic targets for inherited retinal disorders.
- To elucidate the role of specific signaling molecules in retinal health.
- To explore the neuroprotective mechanisms against oxidative damage in the retina.
Main Methods:
- Identification and characterization of a novel signaling molecule, rod-derived cone viability factor (RdCVF).
- Analysis of gene expression and protein products through differential splicing.
- Investigating the function of RdCVF in maintaining photoreceptor cell viability in mouse models.
Main Results:
- RdCVF was identified as a crucial factor for cone photoreceptor cell viability.
- Mice lacking RdCVF showed progressive photoreceptor cell loss.
- A second protein product from the RdCVF gene demonstrated thioredoxin-like activity, protecting photoreceptor and tau proteins from oxidative damage.
Conclusions:
- RdCVF represents a potential therapeutic agent for currently untreatable inherited retinal degenerative diseases.
- The RdCVF signaling pathway may link environmental factors to endogenous neuroprotection.
- Understanding this pathway opens new avenues for treating vision loss associated with retinal degeneration.
