Frataxins Emerge as New Players of the Intracellular Antioxidant Machinery.

ABSTRACT: 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

SUBMITTER: Uceda AB 

PROVIDER: S-EPMC7923443 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature