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Article

The Benefit of Large Neutral Amino Acid Supplementation to a Liberalized Phenylalanine-Restricted Diet in Adult Phenylketonuria Patients: Evidence from Adult Pah-Enu2 Mice

1
Beatrix Children’s Hospital, University Medical Center Groningen, University of Groningen, 9700 RB Groningen, The Netherlands
2
Department of Molecular Neurobiology, Groningen Institute for Evolutionary Life Sciences (GELIFES), University of Groningen, 9700 RB Groningen, The Netherlands
3
Department of Laboratory Medicine, University Medical Center Groningen, University of Groningen, 9700 RB Groningen, The Netherlands
4
Department of Biomedicine and K.G. Jebsen Centre for Neuropsychiatric Disorders, University of Bergen, 5009 Bergen, Norway
*
Author to whom correspondence should be addressed.
Received: 29 August 2019 / Revised: 13 September 2019 / Accepted: 17 September 2019 / Published: 19 September 2019
Many phenylketonuria (PKU) patients cannot adhere to the severe dietary restrictions as advised by the European PKU guidelines, which can be accompanied by aggravated neuropsychological impairments that, at least in part, have been attributed to brain monoaminergic neurotransmitter deficiencies. Supplementation of large neutral amino acids (LNAA) to an unrestricted diet has previously been shown to effectively improve brain monoamines in PKU mice of various ages. To determine the additive value of LNAA supplementation to a liberalized phenylalanine-restricted diet, brain and plasma monoamine and amino acid concentrations in 10 to 16-month-old adult C57Bl/6 PKU mice on a less severe phenylalanine-restricted diet with LNAA supplementation were compared to those on a non-supplemented severe or less severe phenylalanine-restricted diet. LNAA supplementation to a less severe phenylalanine-restricted diet was found to improve both brain monoamine and phenylalanine concentrations. Compared to a severe phenylalanine-restricted diet, it was equally effective to restore brain norepinephrine and serotonin even though being less effective to reduce brain phenylalanine concentrations. These results in adult PKU mice support the idea that LNAA supplementation may enhance the effect of a less severe phenylalanine-restricted diet and suggest that cerebral outcome of PKU patients treated with a less severe phenylalanine-restricted diet may be helped by additional LNAA treatment. View Full-Text
Keywords: phenylketonuria; Pah-enu2 mouse model; dietary treatment; monoaminergic neurotransmitters; large neutral amino acids; adult phenylketonuria; Pah-enu2 mouse model; dietary treatment; monoaminergic neurotransmitters; large neutral amino acids; adult
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MDPI and ACS Style

van Vliet, D.; van der Goot, E.; van Ginkel, W.G.; van Faassen, M.H.J.R.; de Blaauw, P.; Kema, I.P.; Martinez, A.; Heiner-Fokkema, M.R.; van der Zee, E.A.; van Spronsen, F.J. The Benefit of Large Neutral Amino Acid Supplementation to a Liberalized Phenylalanine-Restricted Diet in Adult Phenylketonuria Patients: Evidence from Adult Pah-Enu2 Mice. Nutrients 2019, 11, 2252. https://0-doi-org.brum.beds.ac.uk/10.3390/nu11092252

AMA Style

van Vliet D, van der Goot E, van Ginkel WG, van Faassen MHJR, de Blaauw P, Kema IP, Martinez A, Heiner-Fokkema MR, van der Zee EA, van Spronsen FJ. The Benefit of Large Neutral Amino Acid Supplementation to a Liberalized Phenylalanine-Restricted Diet in Adult Phenylketonuria Patients: Evidence from Adult Pah-Enu2 Mice. Nutrients. 2019; 11(9):2252. https://0-doi-org.brum.beds.ac.uk/10.3390/nu11092252

Chicago/Turabian Style

van Vliet, Danique, Els van der Goot, Wiggert G. van Ginkel, Martijn H.J.R. van Faassen, Pim de Blaauw, Ido P. Kema, Aurora Martinez, M. R. Heiner-Fokkema, Eddy A. van der Zee, and Francjan J. van Spronsen. 2019. "The Benefit of Large Neutral Amino Acid Supplementation to a Liberalized Phenylalanine-Restricted Diet in Adult Phenylketonuria Patients: Evidence from Adult Pah-Enu2 Mice" Nutrients 11, no. 9: 2252. https://0-doi-org.brum.beds.ac.uk/10.3390/nu11092252

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