182 related articles for article (PubMed ID: 24007597)
1. Phenylketonuria: reduced tyrosine brain influx relates to reduced cerebral protein synthesis.
de Groot MJ; Hoeksma M; Reijngoud DJ; de Valk HW; Paans AM; Sauer PJ; van Spronsen FJ
Orphanet J Rare Dis; 2013 Sep; 8():133. PubMed ID: 24007597
[TBL] [Abstract][Full Text] [Related]
2. Large neutral amino acids block phenylalanine transport into brain tissue in patients with phenylketonuria.
Pietz J; Kreis R; Rupp A; Mayatepek E; Rating D; Boesch C; Bremer HJ
J Clin Invest; 1999 Apr; 103(8):1169-78. PubMed ID: 10207169
[TBL] [Abstract][Full Text] [Related]
3. Pharmacologic inhibition of L-tyrosine degradation ameliorates cerebral dopamine deficiency in murine phenylketonuria (PKU).
Harding CO; Winn SR; Gibson KM; Arning E; Bottiglieri T; Grompe M
J Inherit Metab Dis; 2014 Sep; 37(5):735-43. PubMed ID: 24487571
[TBL] [Abstract][Full Text] [Related]
4. Phenylketonuria: brain phenylalanine concentrations relate inversely to cerebral protein synthesis.
de Groot MJ; Sijens PE; Reijngoud DJ; Paans AM; van Spronsen FJ
J Cereb Blood Flow Metab; 2015 Feb; 35(2):200-5. PubMed ID: 25352046
[TBL] [Abstract][Full Text] [Related]
5. Therapeutic brain modulation with targeted large neutral amino acid supplements in the Pah-enu2 phenylketonuria mouse model.
van Vliet D; Bruinenberg VM; Mazzola PN; van Faassen MH; de Blaauw P; Pascucci T; Puglisi-Allegra S; Kema IP; Heiner-Fokkema MR; van der Zee EA; van Spronsen FJ
Am J Clin Nutr; 2016 Nov; 104(5):1292-1300. PubMed ID: 27655443
[TBL] [Abstract][Full Text] [Related]
6. Neurotransmitter positron emission tomographic-studies in adults with phenylketonuria, a pilot study.
Paans AM; Pruim J; Smit GP; Visser G; Willemsen AT; Ullrich K
Eur J Pediatr; 1996 Jul; 155 Suppl 1():S78-81. PubMed ID: 8828616
[TBL] [Abstract][Full Text] [Related]
7. Large Neutral Amino Acid Therapy Increases Tyrosine Levels in Adult Patients with Phenylketonuria: A Long-Term Study.
Burlina AP; Cazzorla C; Massa P; Polo G; Loro C; Gueraldi D; Burlina AB
Nutrients; 2019 Oct; 11(10):. PubMed ID: 31640267
[TBL] [Abstract][Full Text] [Related]
8. Future role of large neutral amino acids in transport of phenylalanine into the brain.
Matalon R; Surendran S; Matalon KM; Tyring S; Quast M; Jinga W; Ezell E; Szucs S
Pediatrics; 2003 Dec; 112(6 Pt 2):1570-4. PubMed ID: 14654667
[TBL] [Abstract][Full Text] [Related]
9. Phenylketonuria oxidative stress and energy dysregulation: Emerging pathophysiological elements provide interventional opportunity.
Dobrowolski SF; Phua YL; Vockley J; Goetzman E; Blair HC
Mol Genet Metab; 2022 Jun; 136(2):111-117. PubMed ID: 35379539
[TBL] [Abstract][Full Text] [Related]
10. Tyrosine metabolism in health and disease: slow-release amino acids therapy improves tyrosine homeostasis in phenylketonuria.
Porta F; Giorda S; Ponzone A; Spada M
J Pediatr Endocrinol Metab; 2020 Dec; 33(12):1519-1523. PubMed ID: 33581706
[TBL] [Abstract][Full Text] [Related]
11. The Effect of Glycomacropeptide versus Amino Acids on Phenylalanine and Tyrosine Variability over 24 Hours in Children with PKU: A Randomized Controlled Trial.
Daly A; Evans S; Chahal S; Santra S; Pinto A; Gingell C; Rocha JC; van Spronsen F; Jackson R; MacDonald A
Nutrients; 2019 Feb; 11(3):. PubMed ID: 30823411
[No Abstract] [Full Text] [Related]
12. The effect of casein glycomacropeptide versus free synthetic amino acids for early treatment of phenylketonuria in a mice model.
Ahring KK; Dagnæs-Hansen F; Brüel A; Christensen M; Jensen E; Jensen TG; Johannsen M; Johansen KS; Lund AM; Madsen JG; Brøndum-Nielsen K; Pedersen M; Sørensen LK; Kjolby M; Møller LB
PLoS One; 2022; 17(1):e0261150. PubMed ID: 35015767
[TBL] [Abstract][Full Text] [Related]
13. Characterization of 2-(methylamino)alkanoic acid capacity to restrict blood-brain phenylalanine transport in Pah enu2 mice: preliminary findings.
Vogel KR; Arning E; Wasek BL; Bottiglieri T; Gibson KM
Mol Genet Metab; 2013; 110 Suppl(0):S71-8. PubMed ID: 23999161
[TBL] [Abstract][Full Text] [Related]
14. Mental health and social functioning in early treated Phenylketonuria: the PKU-COBESO study.
Jahja R; Huijbregts SC; de Sonneville LM; van der Meere JJ; Bosch AM; Hollak CE; Rubio-Gozalbo ME; Brouwers MC; Hofstede FC; de Vries MC; Janssen MC; van der Ploeg AT; Langendonk JG; van Spronsen FJ
Mol Genet Metab; 2013; 110 Suppl():S57-61. PubMed ID: 24183792
[TBL] [Abstract][Full Text] [Related]
15. Glycomacropeptide: long-term use and impact on blood phenylalanine, growth and nutritional status in children with PKU.
Daly A; Evans S; Chahal S; Santra S; Pinto A; Jackson R; Gingell C; Rocha J; Van Spronsen FJ; MacDonald A
Orphanet J Rare Dis; 2019 Feb; 14(1):44. PubMed ID: 30770754
[TBL] [Abstract][Full Text] [Related]
16. The effects of large neutral amino acid supplements in PKU: an MRS and neuropsychological study.
Schindeler S; Ghosh-Jerath S; Thompson S; Rocca A; Joy P; Kemp A; Rae C; Green K; Wilcken B; Christodoulou J
Mol Genet Metab; 2007 May; 91(1):48-54. PubMed ID: 17368065
[TBL] [Abstract][Full Text] [Related]
17. Glycomacropeptide in children with phenylketonuria: does its phenylalanine content affect blood phenylalanine control?
Daly A; Evans S; Chahal S; Santra S; MacDonald A
J Hum Nutr Diet; 2017 Aug; 30(4):515-523. PubMed ID: 28111827
[TBL] [Abstract][Full Text] [Related]
18. Correlations of blood and brain biochemistry in phenylketonuria: Results from the Pah-enu2 PKU mouse.
Dijkstra AM; van Vliet N; van Vliet D; Romani C; Huijbregts SCJ; van der Goot E; Hovens IB; van der Zee EA; Kema IP; Heiner-Fokkema MR; van Spronsen FJ
Mol Genet Metab; 2021 Nov; 134(3):250-256. PubMed ID: 34656426
[TBL] [Abstract][Full Text] [Related]
19. The Impact of a Slow-Release Large Neutral Amino Acids Supplement on Treatment Adherence in Adult Patients with Phenylketonuria.
Burlina AP; Cazzorla C; Massa P; Loro C; Gueraldi D; Burlina AB
Nutrients; 2020 Jul; 12(7):. PubMed ID: 32674279
[TBL] [Abstract][Full Text] [Related]
20. Prefrontal cortex cognitive deficits in children treated early and continuously for PKU.
Diamond A; Prevor MB; Callender G; Druin DP
Monogr Soc Res Child Dev; 1997; 62(4):i-v, 1-208. PubMed ID: 9421921
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]