glutathione synthetase deficiency acidosis

glutathione synthetase deficiency acidosis How Is Diagnosed? Nineteen-year follow-up of a patient

Name: glutathione synthetase deficiency acidosis How Is Diagnosed? Nineteen-year follow-up of a patient
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Nineteen year follow up of a patient with severe glutathione synthetase deficiency Journal of Human Genetics Transsulfuration pathway activation attenuates oxidative stress and ferroptosis in sickle primary erythroblasts and transgenic mice Communications Biology Pyroglutamic acidosis Deranged Physiology Glutathione dysregulation and the etiology and progression of human diseases PMC

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Description

The body then excretes a metal-cysteine mixture

23 , 26972708 (2012) A

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

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