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Gluthathione abnormalities in four children with consistent elevations in multiple acyl-carnitine species. Notice that the patients have lower total (tGSH, μM) and free (fGSH, μM) reduced gluthathione, as well as lower tGSH/fGSSG (free-oxidized gluthathione, μM) and fGSH/fGSSG ratios and higher fGSSG as compared with typically developing controls, suggesting both a reduction in the production of gluthathione and increase in gluthathione utilization by reactive species.
