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  • Futhermore, the PKU model has since been used as a template to shed light on over 200 other inborn errors of metabolism.12,13Biochemistry of PKUPhe exists as d and l enantiomers, and l-Phe is an essential amino acid required for protein synthesis in humans.14 Figure 1 shows the many processes which contribute to the flux of l-Phe in humans.
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