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oai:repisalud.isciii.es:20.500.12105/232712024-11-29T07:08:31Zcom_20.500.12105_15322com_20.500.12105_2051col_20.500.12105_16967

00925njm 22002777a 4500 dc Uceda, Ana Belen author Donoso, Josefa author Frau, Juan author Vilanova, Bartolome author Adrover, Miquel author 2021-02 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 alpha-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. Uceda AB, Donoso J, Frau J, Vilanova B, Adrover M. Frataxins Emerge as New Players of the Intracellular Antioxidant Machinery. Antioxidants. 2021 Feb;10(2):315. https://hdl.handle.net/20.500.13003/19599 https://hdl.handle.net/20.500.12105/23271 33672495 10.3390/antiox10020315 2076-3921 Antioxidants 2-s2.0-85100950798 622033800001 Frataxins Copper Iron Metal-catalyzed oxidation α-synuclein Reactive oxygen species Frataxins Emerge as New Players of the Intracellular Antioxidant Machinery