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Abstracts

Phenylketonuria (PKU)

Phenylketonuria (PKU)

Tyrosine may prevent fetal damage caused by maternal phenylketonuria (PKU). In a study, five maternal PKU pregnancies were observed. Before supplementation, these women had a below normal mean tyrosine plasma concentration of 34 micromole/L, compared to the normal 45 micromole/L. However, with just one dose of 1.3 g L-tyrosine, plasma tyrosine concentrations increased above the recommended concentration of 45 micromole/L. The response was rapid and sustained over a 3-hour study period. The new tyrosine levels significantly increased the ratio of tyrosine to large neutral amino acids. The apparent surplus of tyrosine may readily cross the placenta to provide sufficient amounts to the fetus to support proper protein and catecholamine synthesis.

Rohr, F.J; Lobbregt, D; Levy, H.L.: Tyrosine supplementation in the treatment of maternal phenylketonuria, The-American-journal-of-clinical-nutrition. Mar 1998. v. 67(3) 473-476


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