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The absence of glycine or reduced activity of the enzyme glutathione synthetase leads to accumulation of gamma-glutamylcysteine and its conversion to 5-oxoproline through an alternative pathway

The increase in mitochondrial energy production, which is coupled to the glutamate pool via exchange with alpha ketoglutarate in the mitochondria, and the glutamate glutamine neurotransmitter cycle leads to an increase in neuronal glutamate concentration ( Excitotoxic and ROS Damage During Progression of ALS and Impact on Mitochondrial Energy Production, the Glutamate/Glutamine Neurotransmitter Cycle, and Cellular Concentrations of Glutamate and Glutamine Figure 1C shows the excitotoxic effect of chronic elevated neuronal excitability and glutamate release

Chem Biol Interact 1998;1112:114