Application | Comment | Organism |
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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 | UniProt | Comment | Textmining |
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Homo sapiens | - |
- |
- |