Metabolism, principles glutathione

T19. Tschesche, H., and Macartney, H. W., A new principle of regulation of enzymic activity. Activation and regulation of human polymorphonuclear leukocyte collagenase via disulfide-thiol exchange as catalysed by the glutathione cycle in a peroxidase-coupled reaction to glucose metabolism. Eur. J. Biochem. 120, 183-190 (1981). 

The only disorder of the y-glutamyl cycle for which treatment principles have been developed is glutathione synthetase deficiency (9.2) [1]. The initial symptoms in the neonatal period may be metabolic acidosis and jaundice. Acidosis usually needs to be corrected with sodium bicarbonate, THAM or sodium citrate. Patients may benefit from oral administration of vitamin E (10 mg/kg/day) and vitamin C (100 mg/kg/day). Trials have also been made with N-acetylcysteine and glutathione esters which increased glutathione in leukocytes and plasma. Both these compounds lead to increased intracellular levels of glutathione. However, no decrease in the excretion of 5-oxoprolinuria has been reported. 

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