ReviewJournal of inherited metabolic disease2009
Large neutral amino acids supplementation in phenylketonuric patients.
Review in Journal of inherited metabolic disease, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 44 citations in OpenAlex.
- Current state of the treatment landscape of phenylketonuria.Orphanet journal of rare diseases · 2025Review
- Impact of the gut microbiome composition on social decision-making.PNAS nexus · 2024Article
- The effect of casein glycomacropeptide versus free synthetic amino acids for early treatment of phenylketonuria in a mice model.PloS one · 2022Article
- Nutritional and Metabolic Characteristics of UK Adult Phenylketonuria Patients with Varying Dietary Adherence.Nutrients · 2019Article
- Effect of Blood Phenylalanine Levels on Oxidative Stress in Classical Phenylketonuric Patients.Cellular and molecular neurobiology · 2018Article
- Blood phenylalanine reduction corrects CNS dopamine and serotonin deficiencies and partially improves behavioral performance in adult phenylketonuric mice.Molecular genetics and metabolism · 2018Article
- Evidence of Oxidative Stress and Secondary Mitochondrial Dysfunction in Metabolic and Non-Metabolic Disorders.Journal of clinical medicine · 2017Review
- Protein substitutes for phenylketonuria in Europe: access and nutritional composition.European journal of clinical nutrition · 2016Article
- Dietary intervention in the management of phenylketonuria: current perspectives.Pediatric health, medicine and therapeutics · 2016Review
- Phenylketonuria: a review of current and future treatments.Translational pediatrics · 2015Review
- Phenylketonuria Pathophysiology: on the Role of Metabolic Alterations.Aging and disease · 2015Review
- Phenylketonuria: translating research into novel therapies.Translational pediatrics · 2014Review
- Oxidative stress in phenylketonuria: future directions.Journal of inherited metabolic disease · 2012Review
- Oligodendrocyte development and myelinogenesis are not impaired by high concentrations of phenylalanine or its metabolites.Journal of inherited metabolic disease · 2010Article
- Tetrahydrobiopterin responsiveness after extended loading test of 12 Danish PKU patients with the Y414C mutation.Journal of inherited metabolic disease · 2010Article
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Phenylketonuria is an inborn error of amino acid metabolism that results in severe mental retardation if not treated early and appropriately. The traditional treatment, consisting of a low-phenylalanine diet, is usually difficult to maintain throughout adolescence and adulthood, resulting in undesirable levels of blood phenylalanine and consequent neurotoxicity. The neurotoxicity of phenylalanine is enhanced by its transport mechanism across the blood-brain barrier, which has the highest affinity for phenylalanine compared with the other large neutral amino acids that share the same carrier. The supplementation of large neutral amino acids in phenylketonuric patients has been showing interesting results. Plasma phenylalanine levels can be reduced, which may guarantee important metabolic and clinical benefits to these patients. Although long-term studies are needed to determine the efficacy and safety of large neutral amino acids supplements, the present state of knowledge seems to recommend their prescription to all phenylketonuric adult patients who are non-compliant with the low-phenylalanine diet.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.