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Review

An Overview of the Ferroptosis Hallmarks in Friedreich’s Ataxia

1
Department of Biology, University of Rome Tor Vergata, 00133 Rome, Italy
2
Department of Molecular Medicine and Medical Biotechnologies, University of Naples Federico II, 80131 Naples, Italy
3
IRCCS Fondazione Santa Lucia, 00179 Rome, Italy
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Biomolecules 2020, 10(11), 1489; https://doi.org/10.3390/biom10111489
Received: 22 September 2020 / Revised: 19 October 2020 / Accepted: 26 October 2020 / Published: 28 October 2020
Background: Friedreich’s ataxia (FRDA) is a neurodegenerative disease characterized by early mortality due to hypertrophic cardiomyopathy. FRDA is caused by reduced levels of frataxin (FXN), a mitochondrial protein involved in the synthesis of iron-sulphur clusters, leading to iron accumulation at the mitochondrial level, uncontrolled production of reactive oxygen species and lipid peroxidation. These features are also common to ferroptosis, an iron-mediated type of cell death triggered by accumulation of lipoperoxides with distinct morphological and molecular characteristics with respect to other known cell deaths. Scope of review: Even though ferroptosis has been associated with various neurodegenerative diseases including FRDA, the mechanisms leading to disease onset/progression have not been demonstrated yet. We describe the molecular alterations occurring in FRDA that overlap with those characterizing ferroptosis. Major conclusions: The study of ferroptotic pathways is necessary for the understanding of FRDA pathogenesis, and anti-ferroptotic drugs could be envisaged as therapeutic strategies to cure FRDA. View Full-Text
Keywords: Iron metabolism; FRDA; ROS; neurodegeneration; lipid metabolism; GPX4; frataxin Iron metabolism; FRDA; ROS; neurodegeneration; lipid metabolism; GPX4; frataxin
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MDPI and ACS Style

Turchi, R.; Faraonio, R.; Lettieri-Barbato, D.; Aquilano, K. An Overview of the Ferroptosis Hallmarks in Friedreich’s Ataxia. Biomolecules 2020, 10, 1489. https://doi.org/10.3390/biom10111489

AMA Style

Turchi R, Faraonio R, Lettieri-Barbato D, Aquilano K. An Overview of the Ferroptosis Hallmarks in Friedreich’s Ataxia. Biomolecules. 2020; 10(11):1489. https://doi.org/10.3390/biom10111489

Chicago/Turabian Style

Turchi, Riccardo, Raffaella Faraonio, Daniele Lettieri-Barbato, and Katia Aquilano. 2020. "An Overview of the Ferroptosis Hallmarks in Friedreich’s Ataxia" Biomolecules 10, no. 11: 1489. https://doi.org/10.3390/biom10111489

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