Elisa Cabiscol

This study identifies glial-specific mitochondrial failure and redox imbalance as drivers of regional vulnerability in Friedreich ataxia using a mouse model.

This study shows that frataxin deficiency causes tissue-specific iron homeostasis changes in FXNI151F mice, with brain iron overload and cardiac iron deficiency responses.

This study investigates ferroptosis pathways in Friedreich ataxia models, showing frataxin deficiency disrupts NRF2 activation and leads to oxidative stress and ferroptosis in DRG neurons.

This study shows that frataxin deficiency in DRG neurons reduces mitochondrial function and impairs SirT3 activity, leading to oxidative stress and neuronal damage in FA models.

This study shows that loss of glutathione redox homeostasis impairs proteostasis by inhibiting autophagy-dependent protein degradation in C. elegans and other organisms.