Impaired Activity of the γ-Glutamyl Cycle in Nephropathic Cystinosis Fibroblasts

Abstract

Cystinotic patients have been shown to excrete in their urine high levels of pyroglutamate, an intermediate metabolite of the adenosine triphosphate (ATP)–dependent γ-glutamyl cycle, which is responsible for glutathione (GSH) synthesis. Human fibroblasts were used to study the mechanisms leading to pyroglutamate accumulation in nephropathic cystinosis (NC… (More)
DOI: 10.1203/01.pdr.0000196370.57200.da

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