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Review
. 2023 Apr;43(3):1037-1048.
doi: 10.1007/s10571-022-01243-2. Epub 2022 Jul 6.

Mechanism of Cone Degeneration in Retinitis Pigmentosa

Affiliations
Review

Mechanism of Cone Degeneration in Retinitis Pigmentosa

De-Juan Song et al. Cell Mol Neurobiol. 2023 Apr.

Abstract

Retinitis pigmentosa (RP) is a group of genetic disorders resulting in inherited blindness due to the degeneration of rod and cone photoreceptors. The various mechanisms underlying rod degeneration primarily rely on genetic mutations, leading to night blindness initially. Cones gradually degenerate after rods are almost eliminated, resulting in varying degrees of visual disability and blindness. The mechanism of cone degeneration remains unclear. An understanding of the mechanisms underlying cone degeneration in RP, a highly heterogeneous disease, is essential to develop novel treatments of RP. Herein, we review recent advancements in the five hypotheses of cone degeneration, including oxidative stress, trophic factors, metabolic stress, light damage, and inflammation activation. We also discuss the connection among these theories to provide a better understanding of secondary cone degeneration in RP. Five current mechanisms of cone degenerations in RP Interactions among different pathways are involved in RP.

Keywords: Inflammation; Oxidative stress; RdCVF; Retinal cone photoreceptor cells; Retinitis pigmentosa; Trophic factors.

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Conflict of interest statement

The authors have no relevant financial or non-financial interests to disclose.

Figures

Fig. 1
Fig. 1
The role of oxidative stress in RP
Fig. 2
Fig. 2
The pathways involved in metabolic stress in RP

References

    1. Abed E, Corbo G, Falsini B (2015) Neurotrophin family members as neuroprotectants in retinal degenerations. BioDrugs 29(1):1–13. 10.1007/s40259-014-0110-5 - PubMed
    1. Ait-Ali N, Fridlich R, Millet-Puel G, Clerin E, Delalande F, Jaillard C, Blond F, Perrocheau L, Reichman S, Byrne LC, Olivier-Bandini A, Bellalou J, Moyse E, Bouillaud F, Nicol X, Dalkara D, van Dorsselaer A, Sahel JA, Leveillard T (2015) Rod-derived cone viability factor promotes cone survival by stimulating aerobic glycolysis. Cell 161(4):817–832. 10.1016/j.cell.2015.03.023 - PubMed
    1. Akiyama M, Ikeda Y, Yoshida N, Notomi S, Murakami Y, Hisatomi T, Enaida H, Ishibashi T (2014) Therapeutic efficacy of topical unoprostone isopropyl in retinitis pigmentosa. Acta Ophthalmol 92(3):e229-234. 10.1111/aos.12293 - PubMed
    1. Augsburger F, Filippova A, Rasti D, Seredenina T, Lam M, Maghzal G, Mahiout Z, Jansen-Durr P, Knaus UG, Doroshow J, Stocker R, Krause KH, Jaquet V (2019) Pharmacological characterization of the seven human NOX isoforms and their inhibitors. Redox Biol 26:101272. 10.1016/j.redox.2019.101272 - PMC - PubMed
    1. Birch DG, Weleber RG, Duncan JL, Jaffe GJ, Tao W, Ciliary Neurotrophic Factor Retinitis Pigmentosa Study G (2013) Randomized trial of ciliary neurotrophic factor delivered by encapsulated cell intraocular implants for retinitis pigmentosa. Am J Ophthalmol 156(2):283–292. 10.1016/j.ajo.2013.03.021 - PMC - PubMed

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