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Current Biology , 2012 , 22 , R294-R296

PMID 42009015.

2d1, d2 and 6b, c)

There may be a spectrum of severity, depending on the specific defect in the enzyme and whether there is partial residual function

Then, GS-THT + is metabolized via a -elimination reaction to obtain -glutamyl-dehydroalanyl-glycine (EdAG), which reacts with a second GSH to form lanthionine GSG, a non-reducible analogue of GSH