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FIGURE 3 PEROXIREDOXIN–THIOREDOXIN REDOX CYCLE Peroxiredoxins constitute a family of thiol-specific peroxidases that rely on thioredoxins (Trxs) as the hydrogen donor for the reduction of H 2 O 2 and lipid hydroperoxides ( 2 O 2 produced by the mitochondrial respiratory chain

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In the most severe cases, polymorphisms of GCLC and/or GCLM that result in significantly reduced GCL expression and activity can present with severe phenotype including hemolytic anemia, aminoaciduria and spinocerebellar degeneration [reviewed in 45]