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GSH Quantification Glutathione and GSSG were measured in lysates from cells in 6-well plates as previously described ( ROS Quantification by Spin-Trapping and EPR Spectroscopy Cells (5 10 6 /0.1 ml) were stimulated for 2 h with LPS, then incubated with 50 mM 5-tert-butoxycarbonyl-5-methyl-1-pyrroline-N-oxide (BMPO), synthetized as described previously ( Cell Viability Assay Cells were seeded in 96-well plates at 25,000/well in complete medium

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Particularly, we found that mean plasma levels of Valine (U:140.9 mol/L, K:127.8 mol/L), Lysine (U:109.9 mol/L, K:95.7 mol/L), Threonine (U:67.5 mol/L, K:57.9 mol/L) and Tryptophan (U:2.9 mol/L, K:2.3 mol/L), fell below the expected ranges for healthy adolescents (UCSF Clinical Core Accepted Reference Ranges) for both those affected with konzo and their unaffected siblings