ArticleMolecular and cellular biochemistry2010
Effects of phenylalanine on the survival and neurite outgrowth of rat cortical neurons in primary cultures: possible involvement of brain-derived neurotrophic factor.
Article in Molecular and cellular biochemistry, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 28 citations in OpenAlex.
- Factors associated with psycho-behavioral problems among 100 children with phenylketonuria aged 6-18 years.Orphanet journal of rare diseases · 2025Article
- Neurodegenerative biomarkers and inflammation in patients with propionic and methylmalonic acidemias: effect of L-carnitine treatment.Metabolic brain disease · 2024Article
- Transcriptome Profiling of Phenylalanine-Treated Human Neuronal Model: Spotlight on Neurite Impairment and Synaptic Connectivity.International journal of molecular sciences · 2024Article
- Appropriate dietary phenylalanine improved growth, protein metabolism and lipid metabolism, and glycolysis in largemouth bass (Micropterus salmoides).Fish physiology and biochemistry · 2024Article
- Inborn errors of amino acid metabolism - from underlying pathophysiology to therapeutic advances.Disease models & mechanisms · 2023Article
- A culture model for the assessment of phenylalanine neurotoxicity in phenylketonuria.In vitro models · 2022Article
- The potential neuroprotective effects of stingless bee honey.Frontiers in aging neuroscience · 2022Review
- Prenatal Drugs and Their Effects on the Developing Brain: Insights From Three-Dimensional Human Organoids.Frontiers in neuroscience · 2022Review
- Neurodevelopmental signatures of narcotic and neuropsychiatric risk factors in 3D human-derived forebrain organoids.Molecular psychiatry · 2021Article
- Oxidative stress in phenylketonuria-evidence from human studies and animal models, and possible implications for redox signaling.Metabolic brain disease · 2021Review
- Metabolomics analysis reveals perturbations of cerebrocortical metabolic pathways in the PahCNS neuroscience & therapeutics · 2020Article
- AMP-activated protein kinase activation in mediating phenylalanine-induced neurotoxicity in experimental models of phenylketonuria.Journal of inherited metabolic disease · 2018Article
- Generation of urine-derived induced pluripotent stem cells from a patient with phenylketonuria.Intractable & rare diseases research · 2018Article
- The Behavioral Consequence of Phenylketonuria in Mice Depends on the Genetic Background.Frontiers in behavioral neuroscience · 2016Article
- Doxycycline hinders phenylalanine fibril assemblies revealing a potential novel therapeutic approach in phenylketonuria.Scientific reports · 2015Article
- Altered brain protein expression profiles are associated with molecular neurological dysfunction in the PKU mouse model.Journal of neurochemistry · 2014Article
- The Fas/Fas ligand death receptor pathway contributes to phenylalanine-induced apoptosis in cortical neurons.PloS one · 2013Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Phenylketonuria (PKU) is characterized by elevated levels of phenylalanine (Phe) in plasma and cerebrospinal fluid of PKU patients, leading to mental retardation. The developmental delay in the cerebral cortex is one of the characteristic pathologic changes in untreated phenylketonuria patients. This is thought to be due to the toxic effects of Phe and/or its metabolites; however, the underlying mechanisms are as yet unknown. In this study, using a model system in which cultured cortical neurons were induced with Phe, we observed that Phe inhibited the longest neurite outgrowth and induced the neuronal death. We further demonstrated that the expression of BDNF mRNA and protein was significantly decreased by Phe, together with a decrease in extracellular signal-regulated kinase (ERK) and Akt phosphorylation activity. There was no change in expression of TrkB mRNA and protein. Considering the important role of BDNF in normal brain development and function, these L: -Phe-induced changes in BDNF in PKU brain may be a critical element of the neurological symptoms of PKU.
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