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.

An Overview of the Ferroptosis Hallmarks in Friedreich's Ataxia / Turchi, R.; Faraonio, R.; Lettieri-Barbato, D.; Aquilano, K.. - In: BIOMOLECULES. - ISSN 2218-273X. - 10:11(2020), pp. 1-15. [10.3390/biom10111489]

An Overview of the Ferroptosis Hallmarks in Friedreich's Ataxia

Faraonio R.;
2020

Abstract

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.
2020
An Overview of the Ferroptosis Hallmarks in Friedreich's Ataxia / Turchi, R.; Faraonio, R.; Lettieri-Barbato, D.; Aquilano, K.. - In: BIOMOLECULES. - ISSN 2218-273X. - 10:11(2020), pp. 1-15. [10.3390/biom10111489]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11588/821842
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