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Impaired synthesis of lipoxygenase products in glutathione synthetase deficiency

ABSTRACT: Glutathione synthetase deficiency (GSD) is an inborn error of glutathione (GSH) metabolism leading to a generalized intracellular GSH deficiency. Because GSH is required for leukotriene C4 (LTC4) synthesis, we studied synthesis and metabolism of several lipoxygenase products in two patient...

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Hauptverfasser: Mayatepek, Ertan (VerfasserIn) , Hoffmann, Georg F. (VerfasserIn) , Carlsson, Birgit (VerfasserIn) , Larsson, Agne (VerfasserIn) , Becker, Katja (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: 1994
In: Pediatric research
Year: 1994, Jahrgang: 35, Heft: 3, Pages: 307-310
ISSN:1530-0447
DOI:10.1203/00006450-199403000-00005
Online-Zugang:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1203/00006450-199403000-00005
Verlag, lizenzpflichtig, Volltext: https://www.nature.com/articles/pr1994216
Volltext
Verfasserangaben:Ertan Mayatepek, Georg F. Hoffmann, Birgit Carlsson, Agne Larsson, and Katja Becker

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520 |a ABSTRACT: Glutathione synthetase deficiency (GSD) is an inborn error of glutathione (GSH) metabolism leading to a generalized intracellular GSH deficiency. Because GSH is required for leukotriene C4 (LTC4) synthesis, we studied synthesis and metabolism of several lipoxygenase products in two patients with GSD by radio-HPLC, UV spectrophotometry, and enzyme immunoassays. In both patients, LTC4 synthesis was significantly decreased in calcium ionophore-stimulated neutrophils (up to 0.4 ng/ 106 cells; controls, 5.0 ± 0.9) and monocytes (up to 3.6 ng/ 106 cells; controls, 30.2 ± 3.3). LTB4 synthesis was about seven times higher in GSD cells compared with controls, whereas synthesis of other 5-, 12-, and 15-lipoxygenase products and prostaglandin E2 was not affected. Neutrophils and monocytes from both patients showed a marked reduction in capacity to form [3H]LTC4 from[3H]LTA4 (9-14% of control values). Urinary LTE4 was finally found to be 50-fold lower in GSD, reflecting a decreased synthesis of cysteinyl LT in vivo. GSD may serve as a unique model for the linkage between LT synthesis and GSH metabolism in vivo. 
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