ArticleJournal of inherited metabolic disease2010
Oligodendrocyte development and myelinogenesis are not impaired by high concentrations of phenylalanine or its metabolites.
Article in Journal of inherited metabolic disease, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed, 20 citations in OpenAlex.
- Effects of Sevoflurane on the Development of a Human Brain Microphysiological System.International journal of molecular sciences · 2026Article
- Blood phenylalanine lowering partially reverses white matter changes in a mouse model of phenylketonuria.Molecular therapy : the journal of the American Society of Gene Therapy · 2025Article
- Multi-omics integration reveals Vha68-3 as a testicular aging-specific factor that coordinates spermatid elongation through mitochondrial metabolic homeostasis.Cellular & molecular biology letters · 2025Article
- Neurological and imaging phenotypes of adults with untreated phenylketonuria: new cases and literature review.Journal of neurology · 2023Review
- Genetic etiology and clinical challenges of phenylketonuria.Human genomics · 2022Review
- High phenylalanine concentrations induce demyelination and microglial activation in mouse cerebellar organotypic slices.Frontiers in neuroscience · 2022Article
- Phenylketonuria.Nature reviews. Disease primers · 2021Review
- 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
- Automatic robust neurite detection and morphological analysis of neuronal cell cultures in high-content screening.Neuroinformatics · 2010Article
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Authors and funding
9 authors at 3 institutions in 3 countries.
Funding
Abstract
Phenylketonuria (PKU) is a metabolic genetic disease characterized by deficient phenylalanine hydroxylase (PAH) enzymatic activity. Brain hypomyelination has been reported in untreated patients, but its mechanism remains unclear. We therefore investigated the influence of phenylalanine (Phe), phenylpyruvate (PP), and phenylacetate (PA) on oligodendrocytes. We first showed in a mouse model of PKU that the number of oligodendrocytes is not different in corpus callosum sections from adult mutants or from control brains. Then, using enriched oligodendroglial cultures, we detected no cytotoxic effect of high concentrations of Phe, PP, or PA. Finally, we analyzed the impact of Phe, PP, and PA on the myelination process in myelinating cocultures using both an in vitro index of myelination, based on activation of the myelin basic protein (MBP) promoter, and the direct quantification of myelin sheaths by both optical measurement and a bioinformatics method. None of these parameters was affected by the increased levels of Phe or its derivatives. Taken together, our data demonstrate that high levels of Phe, such as in PKU, are unlikely to directly induce brain hypomyelination, suggesting involvement of alternative mechanisms in this myelination defect.
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