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Glutathione (GSH, / ... This reaction is the rate-limiting step in glutathione synthesis. [3] Second, glycine is added to the C-terminal of γ-glutamylcysteine.
Glutathione synthetase (GSS) (EC 6.3.2.3) is the second enzyme in the glutathione (GSH) biosynthesis pathway. It catalyses the condensation of gamma-glutamylcysteine and glycine, to form glutathione. [2]
Glutamate–cysteine ligase (GCL) EC 6.3.2.2), previously known as γ-glutamylcysteine synthetase (GCS), is the first enzyme of the cellular glutathione (GSH) biosynthetic pathway that catalyzes the chemical reaction:
A second GSH molecule reduces the GS-SeR intermediate back to the selenol, releasing GS-SG as the by-product. A simplified representation is shown below: [5] RSeH + H 2 O 2 → RSeOH + H 2 O RSeOH + GSH → GS-SeR + H 2 O GS-SeR + GSH → GS-SG + RSeH. Glutathione reductase then reduces the oxidized glutathione to complete the cycle:
Glutathione reductase (GR) also known as glutathione-disulfide reductase (GSR) is an enzyme that in humans is encoded by the GSR gene.Glutathione reductase (EC 1.8.1.7) catalyzes the reduction of glutathione disulfide to the sulfhydryl form glutathione (), which is a critical molecule in resisting oxidative stress and maintaining the reducing environment of the cell.
The detoxification reactions comprise the first four steps of mercapturic acid synthesis, [19] with the conjugation to GSH serving to make the substrates more soluble and allowing them to be removed from the cell by transporters such as multidrug resistance-associated protein 1 . [8]
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Glutathione synthetase deficiency (GSD) is a rare autosomal recessive [1] metabolic disorder that prevents the production of glutathione.Glutathione helps prevent damage to cells by neutralizing harmful molecules generated during energy production.