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Literature summary for 3.5.2.9 extracted from

  • Kumar, A.; Bachhawat, A.K.
    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? (2010), J. Biosci., 35, 21-25.
    View publication on PubMed

Application

Application Comment Organism
medicine occurrence of a futile cycle in patients with cystinosis. The enzyme gamma-glutamyl cysteine synthetase, in the absence of cysteine, forms 5-oxoproline instead of the normal substrate, gamma-glutamyl cysteine, and the 5-oxoproline is converted into glutamate by the ATP-dependant enzyme, 5-oxoprolinase. In cysteine-limiting conditions, glutamate is cycled back into glutamate via 5-oxoproline at the cost of two ATP molecules without production of glutathione and is the cause of the decreased levels of glutathione synthesis, as well as the ATP depletion observed in these cells Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
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