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. 2018 Jan 31;83(4):889–896. doi: 10.1038/pr.2017.323

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Copyright © 2018 The Author(s)

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by-nc-nd/4.0/

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Aromatic amino-acid levels in plasma of wild-type (WT) and phenylketonuria (PKU) female mice. Plasma samples, obtained from untreated WT and PKU mice and pegvaliase-treated PKU mice, were analyzed by high-performance liquid chromatography (HPLC) separation. Plasma samples from untreated PKU mice show markedly increased levels in plasma phenylalanine (Phe) relative to WT mice, along with a 40–50% decrease in plasma tyrosine. Plasma tryptophan levels remained similar between the two genotypes. Pegvaliase treatment decreased plasma Phe to physiological or below normal physiological levels according to dose regimen. Tyrosine and tryptophan levels in untreated and pegvaliase-treated PKU mice were similar.