Iron is a key element for mitochondrial function and homeostasis, which is also crucial for
maintaining the neuronal system, but too much iron promotes oxidative stress. A large body …

Cellular iron homeostasis and mitochondrial iron homeostasis are interdependent.
Mitochondria must import iron to form iron–sulfur clusters and heme, and to incorporate …

The indispensable requirement for metals in life processes has led to the evolution of
sophisticated mechanisms that allow organisms to maintain dynamic equilibria of these ions …

The function of frataxin (FXN) has garnered great scientific interest since its depletion was
linked to the incurable neurodegenerative disease Friedreich's ataxia (FRDA). FXN has …

Iron is essential for cell survival and function. It is a transition metal, that could change its
oxidation state from Fe2+ to Fe3+ involving an electron transfer, the key of vital functions but …

Friedreich's ataxia (FRDA) is a rare genetic disorder caused by mutations in the gene
frataxin, encoding for a mitochondrial protein involved in iron handling and in the biogenesis …

Glutathione (GSH) was initially identified and characterized for its redox properties and later
for its contributions to detoxification reactions. Over the past decade, however, the essential …

Iron sulfur (Fe-S) clusters rank among the most ancient and conserved prosthetic groups. Fe-
S clusters containing proteins are present in most, if not all, organisms. Fe-S clusters …

Mitochondrial proteins are encoded by both nuclear and mitochondrial DNA. While some of
the essential subunits of the oxidative phosphorylation (OXPHOS) complexes responsible …

Friedreich's ataxia is a multisystemic genetic disorder within the family of mitochondrial
diseases that is characterized by reduced levels of the essential mitochondrial protein …