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acknowledges support from the NIH (R01NS0433314-14)

Unregulated IV glutathione may cause life-threatening systemic inflammatory response syndrome (SIRS)

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IEM are a class of inborn errors of metabolic pathways that are marked by accumulation (and usually toxic) organic acid metabolites in blood (i.e., organic acidemias) and increased excretion of organic acids in urine (i.e., organic acidurias)

The cystine is added separately at final 0, 5 or 48.3 mg/L (equivalent to 0, 30, and 292 M), depending on experiments

Lu, S.C., et al., Insulin and Glucocorticoid Dependence of Hepatic Gamma-Glutamylcysteine Synthetase and Glutathione Synthesis in the Rat Studies in Cultured-Hepatocytes and Invivo