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Select item 60289451.

Identification of the allosteric site for neutral amino acids in the maize C₄ isozyme of phosphoenolpyruvate carboxylase: The critical role of Ser-100

Lilian González-Segura, Carlos Mújica-Jiménez, Javier Andrés Juárez-Díaz, Rodrigo Güémez-Toro, León P. Martinez-Castilla, Rosario A. Muñoz-Clares

J Biol Chem. 2018 Jun 29; 293(26): 9945–9957. Published online 2018 May 9. doi: 10.1074/jbc.RA118.002884

PMCID:: PMC6028945

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Select item 60543362.

Cooperation of Antiporter LAT2/CD98hc with Uniporter TAT1 for Renal Reabsorption of Neutral Amino Acids

Clara Vilches, Emilia Boiadjieva-Knöpfel, Susanna Bodoy, Simone Camargo, Miguel López de Heredia, Esther Prat, Aida Ormazabal, Rafael Artuch, Antonio Zorzano, François Verrey, Virginia Nunes, Manuel Palacín

J Am Soc Nephrol. 2018 Jun; 29(6): 1624–1635. Published online 2018 Apr 2. doi: 10.1681/ASN.2017111205

PMCID:: PMC6054336

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Select item 72309593.

Large Neutral Amino Acids (LNAAs) Supplementation Improves Neuropsychological Performances in Adult Patients with Phenylketonuria

Iris Scala, Maria Pia Riccio, Maria Marino, Carmela Bravaccio, Giancarlo Parenti, Pietro Strisciuglio

Nutrients. 2020 Apr; 12(4): 1092. Published online 2020 Apr 15. doi: 10.3390/nu12041092

PMCID:: PMC7230959

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Select item 86704854.

Structural basis for substrate specificity of heteromeric transporters of neutral amino acids

Carlos F. Rodriguez, Paloma Escudero-Bravo, Lucía Díaz, Paola Bartoccioni, Carmen García-Martín, Joan G. Gilabert, Jasminka Boskovic, Víctor Guallar, Ekaitz Errasti-Murugarren, Oscar Llorca, Manuel Palacín

Proc Natl Acad Sci U S A. 2021 Dec 7; 118(49): e2113573118. Published online 2021 Nov 30. doi: 10.1073/pnas.2113573118

PMCID:: PMC8670485

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Select item 86181545.

Beneficial Effects of Slow-Release Large Neutral Amino Acids after a Phenylalanine Oral Load in Patients with Phenylketonuria

Iris Scala, Daniela Concolino, Anna Nastasi, Giulia Esposito, Daniela Crisci, Simona Sestito, Stefania Ferraro, Lucia Albano, Margherita Ruoppolo, Giancarlo Parenti, Pietro Strisciuglio

Nutrients. 2021 Nov; 13(11): 4012. Published online 2021 Nov 10. doi: 10.3390/nu13114012

PMCID:: PMC8618154

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Select item 70846846.

Functional Consequences of Low Activity of Transport System A for Neutral Amino Acids in Human Bone Marrow Mesenchymal Stem Cells

Martina Chiu, Giuseppe Taurino, Massimiliano G. Bianchi, Erica Dander, Alessandra Fallati, Nicola Giuliani, Giovanna D’Amico, Ovidio Bussolati

Int J Mol Sci. 2020 Mar; 21(5): 1899. Published online 2020 Mar 10. doi: 10.3390/ijms21051899

PMCID:: PMC7084684

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Select item 74009207.

The Impact of a Slow-Release Large Neutral Amino Acids Supplement on Treatment Adherence in Adult Patients with Phenylketonuria

Alessandro P. Burlina, Chiara Cazzorla, Pamela Massa, Christian Loro, Daniela Gueraldi, Alberto B. Burlina

Nutrients. 2020 Jul; 12(7): 2078. Published online 2020 Jul 14. doi: 10.3390/nu12072078

PMCID:: PMC7400920

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Select item 56146178.

Presumptive brain influx of large neutral amino acids and the effect of phenylalanine supplementation in patients with Tyrosinemia type 1

Willem G. van Ginkel, Danique van Vliet, Johannes G. M. Burgerhof, Pim de Blaauw, M. Estela Rubio Gozalbo, M. Rebecca Heiner-Fokkema, Francjan J. van Spronsen

PLoS One. 2017; 12(9): e0185342. Published online 2017 Sep 26. doi: 10.1371/journal.pone.0185342

PMCID:: PMC5614617

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Select item 52229909.

Long-term treatment of phenylketonuria with a new medical food containing large neutral amino acids

D Concolino, I Mascaro, M T Moricca, G Bonapace, K Matalon, J Trapasso, G Radhakrishnan, C Ferrara, R Matalon, P Strisciuglio

Eur J Clin Nutr. 2017 Jan; 71(1): 51–55. Published online 2016 Sep 14. doi: 10.1038/ejcn.2016.166

Correction in:: Eur J Clin Nutr. 2017; 71(8): 1027.

PMCID:: PMC5222990

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Select item 677059210.

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

Danique van Vliet, Els van der Goot, Wiggert G. van Ginkel, Martijn H. J. R. van Faassen, Pim de Blaauw, Ido P. Kema, Aurora Martinez, M. Rebecca Heiner-Fokkema, Eddy A. van der Zee, Francjan J. van Spronsen

Nutrients. 2019 Sep; 11(9): 2252. Published online 2019 Sep 19. doi: 10.3390/nu11092252

PMCID:: PMC6770592

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Select item 741973511.

Increasing dietary tryptophan in conjunction with decreasing other large neutral amino acids increases weight gain and feed intake in weaner pigs regardless of experimental infection with enterotoxigenic Escherichia coli

Samantha O Sterndale, David W Miller, Josie P Mansfield, Jae C Kim, John R Pluske

J Anim Sci. 2020 Aug; 98(8): skaa190. Published online 2020 Jun 25. doi: 10.1093/jas/skaa190

PMCID:: PMC7419735

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Select item 683550312.

Large Neutral Amino Acid Therapy Increases Tyrosine Levels in Adult Patients with Phenylketonuria: A Long-Term Study

Alessandro P. Burlina, Chiara Cazzorla, Pamela Massa, Giulia Polo, Christian Loro, Daniela Gueraldi, Alberto B. Burlina

Nutrients. 2019 Oct; 11(10): 2541. Published online 2019 Oct 21. doi: 10.3390/nu11102541

PMCID:: PMC6835503

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Select item 654181113.

Meta-regression analysis to predict the influence of branched-chain and large neutral amino acids on growth performance of pigs

Henrique S Cemin, Mike D Tokach, Steve S Dritz, Jason C Woodworth, Joel M DeRouchey, Robert D Goodband

J Anim Sci. 2019 Jun; 97(6): 2505–2514. Published online 2019 Apr 8. doi: 10.1093/jas/skz118

PMCID:: PMC6541811

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Select item 735335914.

Randomized Double-Blind Controlled Trial on the Effect of Proteins with Different Tryptophan/Large Neutral Amino Acid Ratios on Sleep in Adolescents: The PROTMORPHEUS Study

Oussama Saidi, Emmanuelle Rochette, Éric Doré, Freddy Maso, Julien Raoux, Fabien Andrieux, Maria Livia Fantini, Etienne Merlin, Bruno Pereira, Stéphane Walrand, Pascale Duché

Nutrients. 2020 Jun; 12(6): 1885. Published online 2020 Jun 24. doi: 10.3390/nu12061885

PMCID:: PMC7353359

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Select item 299265515.

Large neutral amino acids in the treatment of PKU: from theory to practice

Francjan J. van Spronsen, Martijn J. de Groot, Marieke Hoeksma, Dirk-Jan Reijngoud, Margreet van Rijn

J Inherit Metab Dis. 2010; 33(6): 671–676. Published online 2010 Oct 26. doi: 10.1007/s10545-010-9216-1

PMCID:: PMC2992655

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Select item 948499916.

Aristolochic acid-induced nephropathy is attenuated in mice lacking the neutral amino acid transporter B⁰AT1 (Slc6a19)

Aleix Navarro Garrido, Young Chul Kim, Yuji Oe, Haiyan Zhang, Maria Crespo-Masip, Helen A. Goodluck, Sadhana Kanoo, Paul W. Sanders, Stefan Bröer, Volker Vallon

Am J Physiol Renal Physiol. 2022 Oct 1; 323(4): F455–F467. Published online 2022 Aug 18. doi: 10.1152/ajprenal.00181.2022

PMCID:: PMC9484999

Article

Select item 847875617.

The zebrafish cationic amino acid transporter/glycoprotein-associated family: sequence and spatiotemporal distribution during development of the transport system b^0,+ (slc3a1/slc7a9)

Ståle Ellingsen, Shailesh Narawane, Anders Fjose, Tiziano Verri, Ivar Rønnestad

Fish Physiol Biochem. 2021; 47(5): 1507–1525. Published online 2021 Aug 2. doi: 10.1007/s10695-021-00984-z

PMCID:: PMC8478756

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Select item 466663518.

Large Neutral Amino Acid Supplementation Exerts Its Effect through Three Synergistic Mechanisms: Proof of Principle in Phenylketonuria Mice

Danique van Vliet, Vibeke M. Bruinenberg, Priscila N. Mazzola, Martijn H. J. R. van Faassen, Pim de Blaauw, Ido P. Kema, M. Rebecca Heiner-Fokkema, Rogier D. van Anholt, Eddy A. van der Zee, Francjan J. van Spronsen

PLoS One. 2015; 10(12): e0143833. Published online 2015 Dec 1. doi: 10.1371/journal.pone.0143833

PMCID:: PMC4666635

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Select item 3464719.

Identification and characterization of a lysosomal transporter for small neutral amino acids

Corinne Sagné, Cendra Agulhon, Philippe Ravassard, Michèle Darmon, Michel Hamon, Salah El Mestikawy, Bruno Gasnier, Bruno Giros

Proc Natl Acad Sci U S A. 2001 Jun 19; 98(13): 7206–7211. Published online 2001 Jun 5. doi: 10.1073/pnas.121183498

PMCID:: PMC34647

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Select item 683605220.

Blood and Brain Biochemistry and Behaviour in NTBC and Dietary Treated Tyrosinemia Type 1 Mice

Willem G. van Ginkel, Danique van Vliet, Els van der Goot, Martijn H. J. R. Faassen, Arndt Vogel, M. Rebecca Heiner-Fokkema, Eddy. A. van der Zee, Francjan J. van Spronsen

Nutrients. 2019 Oct; 11(10): 2486. Published online 2019 Oct 16. doi: 10.3390/nu11102486

PMCID:: PMC6836052

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Figure 1.

Proposed model for renal reabsorption of neutral and cationic AAs in mice, showing cooperation of LAT2/CD98hc and TAT1 transporters. Schematic representation of an epithelial cell from the proximal tubule in (A) wild-type and (B) dKO LAT2-TAT1 mice. Arrow head size indicates favored transport direction, and different shape sizes for AAs are used to indicate corresponding concentrations in tubule lumen, cell, and blood. Data presented in this work support a role of TAT1 and LAT2/CD98hc in the basolateral efflux of aromatic and nonaromatic neutral AAs. Ablation of LAT2 and TAT1 resulted in hyperexcretion of neutral and cationic AAs and upregulation of y⁺LAT1/CD98hc heterodimers. The increased intracellular content of neutral and aromatic AAs is hypothesized to compete with cationic AAs for transport via y⁺LAT1/4Fh2 that therefore may contribute to the basolateral efflux of aromatic and nonaromatic neutral AAs. It is also proposed that the increased content of neutral and aromatic AAs might block apical B⁰AT1 and PAT2 transporters. It is speculated that the missing transporter(s) for Pro basolateral efflux might share transport with neutral AAs. Basolateral LAT4 and reversion of basolateral SNAT3 are candidates for efflux of the indicated AA substrates during renal reabsorption. ?, unknown transporter; AA+, cationic amino acids; AA⁰, neutral amino acids; ARO, aromatic amino acids; Collec, collectrin.

Cooperation of Antiporter LAT2/CD98hc with Uniporter TAT1 for Renal Reabsorption of Neutral Amino Acids

J Am Soc Nephrol. 2018 Jun;29(6):1624-1635.
Figure 2

Statistics of types and properties of amino acids in HCC and PCLT. A: Statistics of amino acids types. There was no type of amino acid made distribution discrepancy between HCC samples and PCLT samples. B: Statistics of hydrophobic and hydrophilic amino acids. Numbers of total hydrophobic and hydrophilic amino acids did not make distribution discrepancy between HCC samples and PCLT samples. C: Statistics of acidic, basic and neutral amino acids. Numbers of total acidic, basic and neutral amino acids did not make distribution discrepancy between HCC samples and PCLT samples. D: Statistics of polar and nonpolar amino acids. Numbers of total polar and nonpolar amino acids did not make distribution discrepancy between HCC samples and PCLT samples. E: Statistics of amino acids with special side chains. Numbers of amino acids with special side chains did not make distribution discrepancy between HCC samples and PCLT samples.

Identification of hepatitis B virus X gene variants between hepatocellular carcinoma tissues and pericarcinoma liver tissues in Eastern China

Int J Clin Exp Pathol. 2014;7(9):5988-5996.
Fig. 1

Prescribed large neutral amino acids intake (g/day) on different treatment regimes

Large neutral amino acids in the treatment of PKU: from theory to practice

J Inherit Metab Dis. 2010;33(6):671-676.
Fig 7Comparative changes in levels of amino acids in the mucosa and detrusor following iberiotoxin treatment.

The amino acids are grouped as neutral non-polar, neutral polar, charged positive and charged negative. Essential amino acids are shown in red.

Urothelial MaxiK-activity regulates mucosal and detrusor metabolism

PLoS One. 2017;12(12):e0189387.