Journal article

Publication year: 1994

Pages: 307-310

Journal: Pediatric Research

Volume number: 35

Issue number: 3

ISSN: 0031-3998

eISSN: 1530-0447

DOI Link: https://doi.org/10.1203/00006450-199403000-00005

Publisher: Springer Nature [academic journals on nature.com]

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 C-4 (LTC(4)) 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, LTC(4) synthesis was significantly decreased in calcium ionophore-stimulated neutrophils (up to 0.4 ng/10(6) cells; controls, 5.0 +/- 0.9) and monocytes (up to 3.6 ng/10(6) cells; controls, 30.2 +/- 3.3). LTB(4) synthesis was about seven times higher in GSD cells compared with controls, whereas synthesis of other 5-, 12-, and 15-lipoxygenase products and prostaglandin E(2) was not affected. Neutrophils and monocytes from both patients showed a marked reduction in capacity to form [H-3]LTC(4) from [H-3]LTA(4) (9-14% of control values). Urinary LTE(4) 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.

Harvard Citation style: Mayatepek, E., Hoffmann, G., Carlsson, B., Larsson, A. and Becker, K. (1994) Impaired synthesis of lipoxygenase products in glutathione synthetase deficiency, Pediatric Research, 35(3), pp. 307-310. https://doi.org/10.1203/00006450-199403000-00005

APA Citation style: Mayatepek, E., Hoffmann, G., Carlsson, B., Larsson, A., & Becker, K. (1994). Impaired synthesis of lipoxygenase products in glutathione synthetase deficiency. Pediatric Research. 35(3), 307-310. https://doi.org/10.1203/00006450-199403000-00005