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glutathione synthetase deficiency oxoproline 5-Oxoprolinuria as a cause of high anion gap metabolic acidosis 5 oxoprolinase
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R 1 and R 2 are independently selected from the group consisting of H, linear or branched alkyl (1-25C), aralkyl (6-26C), cycloalkyl (6-25C), heterocycles (6-20C), ethers or polyethers (3-25C), and where R 1 -R 2 together have 2-20C atoms and form a macrocycle with the remainder of formula I
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