Mitigating the pro-oxidant state and melanogenesis of Retinitis pigmentosa: by counteracting mitochondrial dysfunction

Pagano, Giovanni and Pallardó, Federico V. and Lyakhovich, Alex and Tiano, Luca and Trifuoggi, Marco (2021) Mitigating the pro-oxidant state and melanogenesis of Retinitis pigmentosa: by counteracting mitochondrial dysfunction. Cellular and Molecular Life Sciences, 78 (23). pp. 7491-7503. ISSN 1420-682X (Print) 1420-9071 (Online)

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Abstract

Retinitis pigmentosa (RP) is a group of mitochondrial diseases characterized by progressive degeneration of rods and cones leading to retinal loss of light sensitivity and, consequently, to blindness. To date, no cure is available according to the clinical literature. As a disease associated with pigmentation-related, pro-oxidant state, and mitochondrial dysfunction, RP may be viewed at the crossroads of different pathogenetic pathways involved in adverse health outcomes, where mitochondria play a preeminent role. RP has been investigated in a number of experimental and clinical studies aimed at delaying retinal hyperpigmentation by means of a number of natural and synthetic antioxidants, as well as mitochondrial cofactors, also termed mitochondrial nutrients (MNs), such as alpha-lipoic acid, coenzyme Q10 and carnitine. One should consider that each MN plays distinct-and indispensable-roles in mitochondrial function. Thus, a logical choice would imply the administration of MN combinations, instead of individual MNs, as performed in previous studies, and with limited, if any, positive outcomes. A rational study design aimed at comparing the protective effects of MNs, separately or in combinations, and in association with other antioxidants, might foresee the utilization of animal RP models. The results should verify a comparative optimization in preventing or effectively contrasting retinal oxidative stress in mouse RP models and, in prospect, in human RP cases.
Item Type: Article
Additional Information: WoS & Scopus Document Type: Review
Uncontrolled Keywords: Antioxidants; Mitochondrial cofactors; Mitochondrial disease; Oxidative stress; Reactive oxygen species
Divisions: Faculty of Engineering and Natural Sciences
Depositing User: Alex Lyakhovich
Date Deposited: 24 Feb 2022 14:52
Last Modified: 24 Feb 2022 14:52
URI: https://research.sabanciuniv.edu/id/eprint/42757

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