glutathione synthetase deficiency acidosis

glutathione synthetase deficiency acidosis How Is Diagnosed? Pyroglutamic acidosis | Deranged Physiology

Name: glutathione synthetase deficiency acidosis How Is Diagnosed? Pyroglutamic acidosis | Deranged Physiology
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Pyroglutamic acidosis Deranged Physiology Mitochondrial Glutathione in Cellular Redox Homeostasis and Disease Manifestation Frontiers Case report: A Chinese patient with glutathione synthetase deficiency and a novel glutathione synthase mutation Glutathione dysregulation and the etiology and progression of human diseases PMC

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Appl Immunohistochem Mol Morphol AIMM (2018) 26(2):e1521

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EMBO Mol Med

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