Mitigating the pro-oxidant state and melanogenesis of Retinitis pigmentosa: by counteracting mitochondrial dysfunction.
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 pi...
| Autores: | , , , , |
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| Tipo de recurso: | artículo |
| Estado: | Versión publicada |
| Fecha de publicación: | 2021 |
| País: | España |
| Institución: | INCLIVA |
| Repositorio: | r-INCLIVA. Repositorio Institucional de Producción Científica de INCLIVA |
| OAI Identifier: | oai:incliva.fundanetsuite.com:p16102 |
| Acceso en línea: | https://incliva.portalinvestigacion.com/publicaciones/16102 |
| Access Level: | acceso abierto |
| Palabra clave: | Antioxidants Mitochondrial cofactors Mitochondrial disease Oxidative stress Reactive oxygen species |
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Mitigating the pro-oxidant state and melanogenesis of Retinitis pigmentosa: by counteracting mitochondrial dysfunction.Pagano, GiovanniPallardo, Federico VLyakhovich, AlexTiano, LucaTrifuoggi, MarcoAntioxidantsMitochondrial cofactorsMitochondrial diseaseOxidative stressReactive oxygen speciesRetinitis 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.BIRKHAUSER VERLAG AG2021info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://incliva.portalinvestigacion.com/publicaciones/16102CELLULAR AND MOLECULAR LIFE SCIENCESISSN: 1420682XISSNe: 14209071reponame:r-INCLIVA. Repositorio Institucional de Producción Científica de INCLIVAinstname:INCLIVAInglésinfo:eu-repo/semantics/openAccessoai:incliva.fundanetsuite.com:p161022026-06-07T16:35:31Z |
| dc.title.none.fl_str_mv |
Mitigating the pro-oxidant state and melanogenesis of Retinitis pigmentosa: by counteracting mitochondrial dysfunction. |
| title |
Mitigating the pro-oxidant state and melanogenesis of Retinitis pigmentosa: by counteracting mitochondrial dysfunction. |
| spellingShingle |
Mitigating the pro-oxidant state and melanogenesis of Retinitis pigmentosa: by counteracting mitochondrial dysfunction. Pagano, Giovanni Antioxidants Mitochondrial cofactors Mitochondrial disease Oxidative stress Reactive oxygen species |
| title_short |
Mitigating the pro-oxidant state and melanogenesis of Retinitis pigmentosa: by counteracting mitochondrial dysfunction. |
| title_full |
Mitigating the pro-oxidant state and melanogenesis of Retinitis pigmentosa: by counteracting mitochondrial dysfunction. |
| title_fullStr |
Mitigating the pro-oxidant state and melanogenesis of Retinitis pigmentosa: by counteracting mitochondrial dysfunction. |
| title_full_unstemmed |
Mitigating the pro-oxidant state and melanogenesis of Retinitis pigmentosa: by counteracting mitochondrial dysfunction. |
| title_sort |
Mitigating the pro-oxidant state and melanogenesis of Retinitis pigmentosa: by counteracting mitochondrial dysfunction. |
| dc.creator.none.fl_str_mv |
Pagano, Giovanni Pallardo, Federico V Lyakhovich, Alex Tiano, Luca Trifuoggi, Marco |
| author |
Pagano, Giovanni |
| author_facet |
Pagano, Giovanni Pallardo, Federico V Lyakhovich, Alex Tiano, Luca Trifuoggi, Marco |
| author_role |
author |
| author2 |
Pallardo, Federico V Lyakhovich, Alex Tiano, Luca Trifuoggi, Marco |
| author2_role |
author author author author |
| dc.subject.none.fl_str_mv |
Antioxidants Mitochondrial cofactors Mitochondrial disease Oxidative stress Reactive oxygen species |
| topic |
Antioxidants Mitochondrial cofactors Mitochondrial disease Oxidative stress Reactive oxygen species |
| description |
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. |
| publishDate |
2021 |
| dc.date.none.fl_str_mv |
2021 |
| dc.type.none.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
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article |
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publishedVersion |
| dc.identifier.none.fl_str_mv |
https://incliva.portalinvestigacion.com/publicaciones/16102 |
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https://incliva.portalinvestigacion.com/publicaciones/16102 |
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Inglés |
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Inglés |
| dc.rights.none.fl_str_mv |
info:eu-repo/semantics/openAccess |
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openAccess |
| dc.publisher.none.fl_str_mv |
BIRKHAUSER VERLAG AG |
| publisher.none.fl_str_mv |
BIRKHAUSER VERLAG AG |
| dc.source.none.fl_str_mv |
CELLULAR AND MOLECULAR LIFE SCIENCES ISSN: 1420682X ISSNe: 14209071 reponame:r-INCLIVA. Repositorio Institucional de Producción Científica de INCLIVA instname:INCLIVA |
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INCLIVA |
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r-INCLIVA. Repositorio Institucional de Producción Científica de INCLIVA |
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r-INCLIVA. Repositorio Institucional de Producción Científica de INCLIVA |
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1869410589890052096 |
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15.812455 |