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Article

Molecular basis of glutathione synthetase deficiency and a rare gene permutation event.

The EMBO journal - 15 Jun 1999

Polekhina G, Board P G, Gali R R, Rossjohn J, Parker M W

Abstract excerpt

Glutathione synthetase (GS) catalyses the production of glutathione from gamma-glutamylcysteine and glycine in an ATP-dependent manner. Malfunctioning of GS results in disorders including metabolic acidosis, 5-oxoprolinuria, neurological dysfunction, haemolytic anaemia and in some cases is probably lethal. Here we report the crystal structure of human GS (hGS) at 2.1 A resolution in complex with ADP, two...

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Identifiers

PMID: 10369661
DOI: 10.1093/emboj/18.12.3204
PMCID: PMC1171401

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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