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Furthermore, E GSH [IMS] was not affected in a glr1 strain, while E GSH [cytosol] and E GSH [matrix] became significantly more oxidizing than in wt cells

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In CYB5R3-deficient conditions, MetHb builds up, glutathione (GSH) levels rise in response to oxidative stress, and RBCs display morphological changes such as microcytosis, acanthocytosis, and increased membrane fragility ( Due to the lack of mitochondria, RBCs depend on the pentose phosphate pathway (PPP) to produce reducing equivalents in the form of NADPH