FIGURE 3 After 4 h of incubation in the supernatant of intestinal contents of rats, a visible decrease was observed for ASD (M0), while metabolites (M3, M4, and M5) could be detected ( Figure 3B )
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This severe glutathione deficit was accompanied by elevated ventricular lactate, a clear biomarker indicating that the brains of these patients are relying on highly inefficient, oxygen-deprived anaerobic metabolism due to mitochondrial stress
The second step in BH 4 synthesis, catalyzed by 6-pyruvoyltetrahydropterin synthase (PTPS), involves the Zn 2+ and Mg 2+ -dependent rearrangement of 7,8-dihydroneopterin triphosphate, with concommitant loss of the triphosphate, to 6-pyruvoyltetrahydropterin
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