ReviewJournal of inherited metabolic disease2012
Oxidative stress in phenylketonuria: future directions.
Review in Journal of inherited metabolic disease, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers, 1 of them a synthesis that pooled it.
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Who cites it
27 citing papers in PubMed, 1 synthesis or guideline pooled it, 57 citations in OpenAlex.
- Status of nutrients important in brain function in phenylketonuria: a systematic review and meta-analysis.Orphanet journal of rare diseases · 2018Pooled it
- Antioxidant and Anti-Inflammatory Activity of a New Formulation of Slow-Release Amino Acids in Human Intestinal Caco-2 Cells.Antioxidants (Basel, Switzerland) · 2025Article
- Impact of Phenylketonuria on the Serum Metabolome and Plasma Lipidome: A Study in Early-Treated Patients.Metabolites · 2024Article
- Nutrient Status and Intakes of Adults with Phenylketonuria.Nutrients · 2024Article
- L-carnitine protects DNA oxidative damage induced by phenylalanine and its keto acid derivatives in neural cells: a possible pathomechanism and adjuvant therapy for brain injury in phenylketonuria.Metabolic brain disease · 2021Article
- Oxidative stress in phenylketonuria-evidence from human studies and animal models, and possible implications for redox signaling.Metabolic brain disease · 2021Review
- Engineering Organoids forFrontiers in molecular neuroscience · 2021Review
- The cardiovascular phenotype of adult patients with phenylketonuria.Orphanet journal of rare diseases · 2019Article
- Clinical burden of illness in patients with phenylketonuria (PKU) and associated comorbidities - a retrospective study of German health insurance claims data.Orphanet journal of rare diseases · 2019Article
- Newly validated biomarkers of brain damage may shed light into the role of oxidative stress in the pathophysiology of neurocognitive impairment in dietary restricted phenylketonuria patients.Pediatric research · 2019Review
- Assessment of antioxidant enzymes, total sialic acid, lipid bound sialic acid, vitamins and selected amino acids in children with phenylketonuria.Pediatric research · 2018Article
- Article
- Effect of Blood Phenylalanine Levels on Oxidative Stress in Classical Phenylketonuric Patients.Cellular and molecular neurobiology · 2018Article
- Serum ischemia modified albumin is a possible new marker of oxidative stress in phenylketonuria.Metabolic brain disease · 2018Article
- Elevated pentose phosphate pathway is involved in the recovery of hypoxia‑induced erythrocytosis.Molecular medicine reports · 2017Article
- Effect of natural PAL-enzyme on the quality of egg white and mushroom flour and study its impact on the expression of PKU related genes and phenylalanine reduction in mice fed on.Journal, genetic engineering & biotechnology · 2017Article
- Evidence of Oxidative Stress and Secondary Mitochondrial Dysfunction in Metabolic and Non-Metabolic Disorders.Journal of clinical medicine · 2017Review
- Supplementation of Micronutrient Selenium in Metabolic Diseases: Its Role as an Antioxidant.Oxidative medicine and cellular longevity · 2017Review
- Nutritional status of patients with phenylketonuria in Japan.Molecular genetics and metabolism reports · 2016Article
- Protein substitutes for phenylketonuria in Europe: access and nutritional composition.European journal of clinical nutrition · 2016Article
Corrections and comments
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Authors and funding
2 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Phenylketonuria represents the most prevalent inborn error of amino acid metabolism. In early diagnosed patients adequate and continued dietary treatment results in a good neurologic outcome. Natural protein and phenylalanine-restricted diet, even if rich in fruits and vegetables, represents a serious risk for nutritional deficiencies, albeit universally accepted. In the last few years, a growing number of reports have been describing oxidative stress as a concern in phenylketonuric patients. The diet itself includes good sources of dietary antioxidants (phytochemicals, some vitamins and minerals) but also a risk factor for some deficiencies (selenium, zinc, ubiquinone-10 and L-carnitine). Additionally, the extreme stringency of the diet may impose a reduced synthesis of endogenous antioxidants (like ubiquinone-10 and glutathione). Furthermore, increased phenylalanine levels, and its metabolites, may enhance the endogenous synthesis of reactive species and free radicals and/or interfere with the endogenous synthesis of enzymatic antioxidants (like glutathione peroxidase). Therefore, oxidative stress will probably increase, mainly in late diagnosed patients or in those with bad metabolic control. Considering the known association between oxidative stress, obesity and cardiovascular disease, it seems advisable to look further to the impact of oxidative stress on body macromolecules and structures (like lipoprotein oxidation), especially in phenylketonuric patients with late diagnosis or bad metabolic control, in order to prevent future increased risks. Recommendations for PKU patient's clinical follow-up improvement and educational goals are included.
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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.