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Among the most significant metabolites accumulated in FH-deficient cells a putative GSH adduct was detected (annotated by Metlin database as the formate adduct of pyruvilGSH
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First, because the last enzyme (GLDH enzyme) of the L-galactose pathway is a component of the mitochondrial complex I, thus its catalytic activity could be regulated by the electron flow across this protein complex
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Pallafacchina, G
