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Next, electrons supplied by the cofactor nicotinamide adenine dinucleotide phosphate (NADPH) are transferred to the CYP by two intramembrane proteins, cytochrome P450 reductase and cytochrome b5, reducing the heme iron to the ferrous (Fe 2+ ) state

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The blood ammonia levels in children with GDH-HI are not as high as Urea cycle disorder (UCD) patients, and the blood ammonia level is stable