
Fig. 1.
Enforced metabolic pathways of accumulated l-Phe and l-Tyr in a person suffering from PKU and type I tyrosinemia diseases.

Fig. 2.
Alternative metabolic pathways of l-Tyr in a person suffering from type II tyrosinemia disease.

Fig. 3.
Reversible conversion of l-Phe and l-Tyr into corresponding oxoacids catalyzed by the enzyme PheDH.
Table 1.
Solvent isotope effects (SIE) and kinetic isotope effects (KIE) in the oxidative deamination of l-Tyr and reductive amination of PPA catalyzed by enzyme PheDH
| Reactant | SIE | |
|---|---|---|
| on Vmax | on Vmax/KM | |
| l-Tyr | 1.43 ± 0.09 | 1.48 ± 0.12 |
| PPA | 1.44 ± 0.08 | 1.58 ± 0.14 |
| on Vmax | on Vmax/KM | |
| l-Tyr/[2-2H]-l-Tyr | 2.26 ± 0.10 | 2.87 ± 0.11 |
| PPA/[(3S)-2H]-PPA | 1.55 ± 0.06 | 1.53 ± 0.16 |