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The supply pathway involves the export of GSH from astrocytes via the multidrugresistance protein 1 (Mrp1), extracellular processing of GSH by astrocytic glutamyl transpeptidase (GT) to the dipeptide cysteinylglycine (CysGly) and hydrolysis of this dipeptide by neuronal aminopeptidase N (ApN) to cysteine (Cys) and glycine (Gly)
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Brasell EJ, Chu L, El Kares R, Seo JH, Loesch R, Iglesias DM, Goodyer P (2019) The aminoglycoside geneticin permits translational readthrough of the CTNS W138X nonsense mutation in fibroblasts from patients with nephropathic cystinosis