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Atp13A2 deficiency disrupts lysosomal polyamine export

One specific enzyme, called gamma-glutamyl transpeptidase, is particularly good at breaking glutathione down into its individual amino acid components

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Because the body synthesizes glutathione where it is needed and compartmentalizes it deliberately, simply raising the concentration of glutathione in the bloodstream does not guarantee that any particular tissue compartment least of all a target as specific as the melanocytes in the basal epidermis sees a meaningful change

[DOI] [PMC free article] [PubMed] [Google Scholar] 44.Grozio A., Mills K.F., Yoshino J., Bruzzone S., Sociali G., Tokizane K., Lei H.C., Cunningham R., Sasaki Y., Migaud M.E., et al

In addition, both ADPKD cell and mouse models displayed high iron content, low GPX4 activity, increased lipid peroxide accumulation, and a tendency toward ferroptosis