Ferroptosis in Friedreich’s Ataxia: A Metal-Induced Neurodegenerative Disease
Ferroptosis is an iron-dependent form of regulated cell death, arising from the accumulation of lipid-based reactive oxygen species when glutathione-dependent repair systems are compromised. Lipid peroxidation, mitochondrial impairment and iron dyshomeostasis are the hallmark of ferroptosis, which i...
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| Format: | Article |
| Language: | English |
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MDPI AG
2020-11-01
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| Series: | Biomolecules |
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| Online Access: | https://www.mdpi.com/2218-273X/10/11/1551 |
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| author | Piergiorgio La Rosa Sara Petrillo Maria Teresa Fiorenza Enrico Silvio Bertini Fiorella Piemonte |
| author_facet | Piergiorgio La Rosa Sara Petrillo Maria Teresa Fiorenza Enrico Silvio Bertini Fiorella Piemonte |
| author_sort | Piergiorgio La Rosa |
| collection | DOAJ |
| description | Ferroptosis is an iron-dependent form of regulated cell death, arising from the accumulation of lipid-based reactive oxygen species when glutathione-dependent repair systems are compromised. Lipid peroxidation, mitochondrial impairment and iron dyshomeostasis are the hallmark of ferroptosis, which is emerging as a crucial player in neurodegeneration. This review provides an analysis of the most recent advances in ferroptosis, with a special focus on Friedreich’s Ataxia (FA), the most common autosomal recessive neurodegenerative disease, caused by reduced levels of frataxin, a mitochondrial protein involved in iron–sulfur cluster synthesis and antioxidant defenses. The hypothesis is that the iron-induced oxidative damage accumulates over time in FA, lowering the ferroptosis threshold and leading to neuronal cell death and, at last, to cardiac failure. The use of anti-ferroptosis drugs combined with treatments able to activate the antioxidant response will be of paramount importance in FA therapy, such as in many other neurodegenerative diseases triggered by oxidative stress. |
| first_indexed | 2024-03-10T14:52:06Z |
| format | Article |
| id | doaj.art-b594995121754060be945c914acba8fe |
| institution | Directory Open Access Journal |
| issn | 2218-273X |
| language | English |
| last_indexed | 2024-03-10T14:52:06Z |
| publishDate | 2020-11-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biomolecules |
| spelling | doaj.art-b594995121754060be945c914acba8fe2023-11-20T20:54:54ZengMDPI AGBiomolecules2218-273X2020-11-011011155110.3390/biom10111551Ferroptosis in Friedreich’s Ataxia: A Metal-Induced Neurodegenerative DiseasePiergiorgio La Rosa0Sara Petrillo1Maria Teresa Fiorenza2Enrico Silvio Bertini3Fiorella Piemonte4Department of Psychology, Division of Neuroscience, Sapienza University of Rome, 00185 Rome, ItalyUnit of Muscular and Neurodegenerative Diseases, Bambino Gesù Children’s Hospital, IRCCS, 00146 Rome, ItalyDepartment of Psychology, Division of Neuroscience, Sapienza University of Rome, 00185 Rome, ItalyUnit of Muscular and Neurodegenerative Diseases, Bambino Gesù Children’s Hospital, IRCCS, 00146 Rome, ItalyUnit of Muscular and Neurodegenerative Diseases, Bambino Gesù Children’s Hospital, IRCCS, 00146 Rome, ItalyFerroptosis is an iron-dependent form of regulated cell death, arising from the accumulation of lipid-based reactive oxygen species when glutathione-dependent repair systems are compromised. Lipid peroxidation, mitochondrial impairment and iron dyshomeostasis are the hallmark of ferroptosis, which is emerging as a crucial player in neurodegeneration. This review provides an analysis of the most recent advances in ferroptosis, with a special focus on Friedreich’s Ataxia (FA), the most common autosomal recessive neurodegenerative disease, caused by reduced levels of frataxin, a mitochondrial protein involved in iron–sulfur cluster synthesis and antioxidant defenses. The hypothesis is that the iron-induced oxidative damage accumulates over time in FA, lowering the ferroptosis threshold and leading to neuronal cell death and, at last, to cardiac failure. The use of anti-ferroptosis drugs combined with treatments able to activate the antioxidant response will be of paramount importance in FA therapy, such as in many other neurodegenerative diseases triggered by oxidative stress.https://www.mdpi.com/2218-273X/10/11/1551ferroptosisFriedreich’s Ataxiaironneurodegenerationoxidative stress |
| spellingShingle | Piergiorgio La Rosa Sara Petrillo Maria Teresa Fiorenza Enrico Silvio Bertini Fiorella Piemonte Ferroptosis in Friedreich’s Ataxia: A Metal-Induced Neurodegenerative Disease Biomolecules ferroptosis Friedreich’s Ataxia iron neurodegeneration oxidative stress |
| title | Ferroptosis in Friedreich’s Ataxia: A Metal-Induced Neurodegenerative Disease |
| title_full | Ferroptosis in Friedreich’s Ataxia: A Metal-Induced Neurodegenerative Disease |
| title_fullStr | Ferroptosis in Friedreich’s Ataxia: A Metal-Induced Neurodegenerative Disease |
| title_full_unstemmed | Ferroptosis in Friedreich’s Ataxia: A Metal-Induced Neurodegenerative Disease |
| title_short | Ferroptosis in Friedreich’s Ataxia: A Metal-Induced Neurodegenerative Disease |
| title_sort | ferroptosis in friedreich s ataxia a metal induced neurodegenerative disease |
| topic | ferroptosis Friedreich’s Ataxia iron neurodegeneration oxidative stress |
| url | https://www.mdpi.com/2218-273X/10/11/1551 |
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