Cargando…

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

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. Sup...

Descripción completa

Detalles Bibliográficos
Autores principales:	van Vliet, Danique, van der Goot, Els, van Ginkel, Wiggert G., van Faassen, Martijn H. J. R., de Blaauw, Pim, Kema, Ido P., Martinez, Aurora, Heiner-Fokkema, M. Rebecca, van der Zee, Eddy A., van Spronsen, Francjan J.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2019
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6770592/ https://www.ncbi.nlm.nih.gov/pubmed/31546852 http://dx.doi.org/10.3390/nu11092252

Ejemplares similares

Gut-Microbiome Composition in Response to Phenylketonuria Depends on Dietary Phenylalanine in BTBR Pah(enu2) Mice
por: van der Goot, Els, et al.
Publicado: (2022)

The Behavioral Consequence of Phenylketonuria in Mice Depends on the Genetic Background
por: Bruinenberg, Vibeke M., et al.
Publicado: (2016)

Dried blood spot versus venous blood sampling for phenylalanine and tyrosine
por: van Vliet, Kimber, et al.
Publicado: (2020)

Large Neutral Amino Acid Supplementation Exerts Its Effect through Three Synergistic Mechanisms: Proof of Principle in Phenylketonuria Mice
por: van Vliet, Danique, et al.
Publicado: (2015)

Presumptive brain influx of large neutral amino acids and the effect of phenylalanine supplementation in patients with Tyrosinemia type 1
por: van Ginkel, Willem G., et al.
Publicado: (2017)

Blood and Brain Biochemistry and Behaviour in NTBC and Dietary Treated Tyrosinemia Type 1 Mice
por: van Ginkel, Willem G., et al.
Publicado: (2019)

Long-term dietary intervention with low Phe and/or a specific nutrient combination improve certain aspects of brain functioning in phenylketonuria (PKU)
por: Bruinenberg, Vibeke M., et al.
Publicado: (2019)

Biomarkers of Micronutrients in Regular Follow-Up for Tyrosinemia Type 1 and Phenylketonuria Patients
por: van Vliet, Kimber, et al.
Publicado: (2019)

The Effect of Various Doses of Phenylalanine Supplementation on Blood Phenylalanine and Tyrosine Concentrations in Tyrosinemia Type 1 Patients
por: van Ginkel, Willem G., et al.
Publicado: (2019)

A Specific Nutrient Combination Attenuates the Reduced Expression of PSD-95 in the Proximal Dendrites of Hippocampal Cell Body Layers in a Mouse Model of Phenylketonuria
por: Bruinenberg, Vibeke M., et al.
Publicado: (2016)

Sleep Disturbances in Phenylketonuria: An Explorative Study in Men and Mice
por: Bruinenberg, Vibeke M., et al.
Publicado: (2017)

Metabolomics analysis reveals perturbations of cerebrocortical metabolic pathways in the Pah(enu2) mouse model of phenylketonuria
por: Lu, Li‐Hua, et al.
Publicado: (2019)

Monitoring phenylalanine concentrations in the follow‐up of phenylketonuria patients: An inventory of pre‐analytical and analytical variation
por: Coene, Karlien L. M., et al.
Publicado: (2020)

Single amino acid supplementation in aminoacidopathies: a systematic review
por: van Vliet, Danique, et al.
Publicado: (2014)

Bone mineral density is within normal range in most adult phenylketonuria patients
por: Lubout, Charlotte M. A., et al.
Publicado: (2020)

A Microbial Community Ecology Perspective on the Gut-Microbiome-Brain Axis
por: van der Goot, Els, et al.
Publicado: (2020)

Optimising amino acid absorption: essential to improve nitrogen balance and metabolic control in phenylketonuria
por: MacDonald, Anita, et al.
Publicado: (2018)

Physiological competition of brain phenylalanine accretion: Initial pharmacokinetic analyses of aminoisobutyric and methylaminoisobutyric acids in Pah(enu2 −/−) mice
por: Vogel, Kara R., et al.
Publicado: (2015)

Creatine energy substrate increases bone density in the Pah(enu2) classical PKU mouse in the context of phenylalanine restriction
por: Dobrowolski, Steven F., et al.
Publicado: (2023)

Sex effects of dietary protein source and acid load on renal and bone status in the Pah(enu2) mouse model of phenylketonuria
por: Stroup, Bridget M., et al.
Publicado: (2019)

Aspartame and Phe-Containing Degradation Products in Soft Drinks across Europe
por: van Vliet, Kimber, et al.
Publicado: (2020)

Long-Term Follow-Up of Cognition and Mental Health in Adult Phenylketonuria: A PKU-COBESO Study
por: Jahja, Rianne, et al.
Publicado: (2017)

Glutamine energy substrate anaplerosis increases bone density in the Pah(enu2) classical PKU mouse in the absence of phenylalanine restriction
por: Dobrowolski, Steven F., et al.
Publicado: (2022)

Genetically engineered probiotic for the treatment of phenylketonuria (PKU); assessment of a novel treatment in vitro and in the PAH(enu2) mouse model of PKU
por: Durrer, Katherine E., et al.
Publicado: (2017)

Tetrahydrobiopterin responsiveness in phenylketonuria: prediction with the 48-hour loading test and genotype
por: Anjema, Karen, et al.
Publicado: (2013)

The neonatal tetrahydrobiopterin loading test in phenylketonuria: what is the predictive value?
por: Anjema, Karen, et al.
Publicado: (2016)

The cardiovascular phenotype of adult patients with phenylketonuria
por: Azabdaftari, Aline, et al.
Publicado: (2019)

Dietary Liberalization in Tetrahydrobiopterin-Treated PKU Patients: Does It Improve Outcomes?
por: Evers, Roeland A. F., et al.
Publicado: (2022)

Long-Term Outcomes and Practical Considerations in the Pharmacological Management of Tyrosinemia Type 1
por: van Ginkel, Willem G., et al.
Publicado: (2019)

Improved attention linked to sustained phenylalanine reduction in adults with early‐treated phenylketonuria
por: Bilder, Deborah A., et al.
Publicado: (2021)

Phenylketonuria: reduced tyrosine brain influx relates to reduced cerebral protein synthesis
por: de Groot, Martijn J, et al.
Publicado: (2013)

Preventive use of nitisinone in alkaptonuria
por: Wolffenbuttel, Bruce H. R., et al.
Publicado: (2021)

Daily variation of NTBC and its relation to succinylacetone in tyrosinemia type 1 patients comparing a single dose to two doses a day
por: Kienstra, Nienke S., et al.
Publicado: (2017)

Phenylalanine Tolerance over Time in Phenylketonuria: A Systematic Review and Meta-Analysis
por: Pinto, Alex, et al.
Publicado: (2023)

Age-Related Psychophysiological Vulnerability to Phenylalanine in Phenylketonuria
por: Leuzzi, Vincenzo, et al.
Publicado: (2014)

Acute exercise in treated phenylketonuria patients: Physical activity and biochemical response
por: Mazzola, Priscila Nicolao, et al.
Publicado: (2015)

Parenting a child with phenylketonuria or galactosemia: implications for health-related quality of life
por: ten Hoedt, Amber E., et al.
Publicado: (2011)

Mild hyperphenylalaninemia: to treat or not to treat
por: van Spronsen, Francjan J.
Publicado: (2011)

Metabolomic Insights into the Nutritional Status of Adults and Adolescents with Phenylketonuria Consuming a Low-Phenylalanine Diet in Combination with Amino Acid and Glycomacropeptide Medical Foods
por: Stroup, Bridget M., et al.
Publicado: (2017)

Therapeutic implication of L-phenylalanine aggregation mechanism and its modulation by D-phenylalanine in phenylketonuria
por: Singh, Virender, et al.
Publicado: (2014)

Cannot write session to /tmp/vufind_sessions/sess_nkmrnuk7dnugamtddlpiha9iak