glutathione synthetase deficiency acidosis

glutathione synthetase deficiency acidosis How Is Diagnosed? Mitochondrial Glutathione in Cellular Redox

Name: glutathione synthetase deficiency acidosis How Is Diagnosed? Mitochondrial Glutathione in Cellular Redox
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Mitochondrial Glutathione in Cellular Redox Homeostasis and Disease Manifestation Minireview on Glutamine Synthetase Deficiency, an Ultra Rare Inborn Error of Amino Acid Biosynthesis Glutathione dysregulation and the etiology and progression of human diseases PMC Frontiers Case report: A Chinese patient with glutathione synthetase deficiency and a novel glutathione synthase mutation Pyroglutamic acidosis Deranged Physiology

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World J Diabetes 2021;12:124-37

Since reduced glutathione has a half-life of around 9 hours at pH 7.5 in the absence of metals (Stevens et al., 1983) and multiple times faster in the presence of certain metals (Voegtlin et al., 1931), we wanted to exclude that GSH oxidizes over time in our assay, resulting in the formation of GSSG