Frataxin
2022-05-30: reference:
Frataxin #
Mostly in Mitochondria. Iron chaperone/storage protein that mediates iron clustering with sulfur.
- Defective frataxin = Friedreich ataxia: mitochondrial iron overload, leading to ROS and sensitivity to oxiative stress.
- Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis