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A futile cycle, formed between two ATP-dependant gamma-glutamyl cycle enzymes, gamma-glutamyl cysteine synthetase and 5-oxoprolinase: the cause of cellular ATP depletion in nephrotic cystinosis

6-MP undergoes two major inactivation routes

doi: 10.1007/s00425-023-04174-4 21 HeK.SongS.ZouZ.FengM.WangD.WangY.et al

L-Glutathione (reduced) - 500 mg

Ketone bodies are non-fermentable and can reduce GBM acidification As fermentation metabolism is ultimately responsible for rapid GBM growth and the acidification of the microenvironment, then therapies that target fermentation metabolism should reduce acidification and GBM growth