Phenylketonuria

Phenylketonuria (PKU) is treated by restricting dietary intake of natural protein and substituting a protein source that lacks phenylalanine but is fortified with tyrosine. Tyrosine 1172

This recommendation is because people with PKU are unable to metabolise

phenylalanine, the precursor to tyrosine. Unfortunately, tyrosine supplementation has not been shown to consistently improve neuropsychologic function in PKU, which is possibly because increases in plasma tyrosine levels are not sustained and brain influx often remains suboptimal despite tyrosine supplementation (Kalsner et al 2001). In practice, plasma tyrosine levels are monitored and controlled (normal: 45 micromol/L) before tyrosine supplementation is considered (Poustie & Rutherford 2000, van Spronsen et al 2001).

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