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Article

Frataxins Emerge as New Players of the Intracellular Antioxidant Machinery

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Departament de Química, Institut Universitari d’Investigació en Ciències de la Salut (IUNICS), Universitat de les Illes Balears, Ctra. Valldemossa km 7.5, E-07122 Palma de Mallorca, Spain
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Institut d’Investigació Sanitària de les Illes Balears (IdISBa), E-07120 Palma de Mallorca, Spain
*
Author to whom correspondence should be addressed.
Academic Editor: Claus Jacob
Antioxidants 2021, 10(2), 315; https://doi.org/10.3390/antiox10020315
Received: 31 December 2020 / Revised: 17 February 2021 / Accepted: 18 February 2021 / Published: 20 February 2021
(This article belongs to the Collection Feature Paper in ROS, RNS, RSS)
Frataxin is a mitochondrial protein which deficiency causes Friedreich’s ataxia, a cardio-neurodegenerative disease. The lack of frataxin induces the dysregulation of mitochondrial iron homeostasis and oxidative stress, which finally causes the neuronal death. The mechanism through which frataxin regulates the oxidative stress balance is rather complex and poorly understood. While the absence of human (Hfra) and yeast (Yfh1) frataxins turn out cells sensitive to oxidative stress, this does not occur when the frataxin gene is knocked-out in E. coli. To better understand the biological roles of Hfra and Yfh1 as endogenous antioxidants, we have studied their ability to inhibit the formation of reactive oxygen species (ROS) from Cu2+- and Fe3+-catalyzed degradation of ascorbic acid. Both proteins drastically reduce the formation of ROS, and during this process they are not oxidized. In addition, we have also demonstrated that merely the presence of Yfh1 or Hfra is enough to protect a highly oxidation-prone protein such as α-synuclein. This unspecific intervention (without a direct binding) suggests that frataxins could act as a shield to prevent the oxidation of a broad set of intracellular proteins, and reinforces that idea that frataxin can be used to prevent neurological pathologies linked to an enhanced oxidative stress. View Full-Text
Keywords: frataxins; copper; iron; metal-catalyzed oxidation; α-synuclein; reactive oxygen species frataxins; copper; iron; metal-catalyzed oxidation; α-synuclein; reactive oxygen species
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MDPI and ACS Style

Uceda, A.B.; Donoso, J.; Frau, J.; Vilanova, B.; Adrover, M. Frataxins Emerge as New Players of the Intracellular Antioxidant Machinery. Antioxidants 2021, 10, 315. https://doi.org/10.3390/antiox10020315

AMA Style

Uceda AB, Donoso J, Frau J, Vilanova B, Adrover M. Frataxins Emerge as New Players of the Intracellular Antioxidant Machinery. Antioxidants. 2021; 10(2):315. https://doi.org/10.3390/antiox10020315

Chicago/Turabian Style

Uceda, Ana B., Josefa Donoso, Juan Frau, Bartolomé Vilanova, and Miquel Adrover. 2021. "Frataxins Emerge as New Players of the Intracellular Antioxidant Machinery" Antioxidants 10, no. 2: 315. https://doi.org/10.3390/antiox10020315

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