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& Langin, D

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Most of the patients with rare diseases receive treatments intended to alleviate the disease-derived complications and improve their quality of life, but without tackling the underlying disease cause

Inhibition of the Xc-system determines the decrease in GSH levels, which indirectly leads to the inactivation of GPX4 and the onset of ferroptosis ( 3+ enters cells with the assistance of transferrin and subsequently converts to Fe 2+ ( 2+ can catalyze the generation of metabolically toxic reactive oxygen species and phospholipid peroxide through the Fenton reaction, leading to cell damage or death (Figure 4)