ArticleNature chemical biology2012
Phenylalanine assembly into toxic fibrils suggests amyloid etiology in phenylketonuria.
Article in Nature chemical biology, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 134 papers.
What it found
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
134 citing papers in PubMed, 461 citations in OpenAlex.
- Transient brain structure changes after high phenylalanine exposure in adults with phenylketonuria.Brain : a journal of neurology · 2024Trial
- Functional abiotic metabolite assembly hypothesis for the early events in the origin of life.Nature communications · 2026Review
- Shikimate pathway disruption in yeast induces metabolite self-assembly into toxic aggregates.The FEBS journal · 2026Article
- Chirality Effect on Physical and Biological Properties of Peptide-Based Hydrogels.Gels (Basel, Switzerland) · 2026Review
- Self-assembled metabolite-derived amyloid-like structures: implications for disease, therapeutics, and biomedical applications.Molecular biology reports · 2026Review
- Phenylalanine homeostasis in metabolic disorders: epidemiological trends, pathophysiological mechanisms, and clinical treatment.Frontiers in endocrinology · 2026Review
- Cathepsin C-Catalyzed Ligation Generates Intralysosomal Amyloid Fibrils from Dipeptide Esters.bioRxiv : the preprint server for biology · 2025Article
- Allopurinol Reprograms Uric Acid Self-Assembly by Disrupting Cytotoxic Fibrils and Redirecting Crystal Growth.JACS Au · 2025Article
- Unveiling the phenylalanine coaggregation mechanism for a deep understanding of phenylketonuria disease.Scientific reports · 2025Article
- Controlling Amyloid Assembly Dynamics Using Spin Interfaces.ACS nano · 2025Article
- Spectroscopic Investigation of Hydrogels Formed from Phenylalanine.Journal of fluorescence · 2025Article
- Article
- Experimental Animal Models of Phenylketonuria: Pros and Cons.International journal of molecular sciences · 2025Review
- Oxidative stress induced protein aggregation via GGCT produced pyroglutamic acid in drug resistant glioblastoma.iScience · 2025Article
- Ordered Aggregates of Fmoc-Diphenylalanine at Alkaline pH as a Precursor of Fibril Formation and Peptide Gelation.The journal of physical chemistry. B · 2025Article
- Phe-Phe-Based Macroscopic Supramolecular Hydrogel Construction Strategies and Biomedical Applications.Chem & bio engineering · 2024Review
- Cavity Lasing of Thioflavin T in the Condensed Phase for Discrimination between Surface Interaction and β-Sheet Groove Binding in Alzheimer-Linked Peptides.The journal of physical chemistry letters · 2024Article
- The rise and fall of adenine clusters in the gas phase: a glimpse into crystal growth and nucleation.Analytical and bioanalytical chemistry · 2024Article
- Amylum forms typical self-assembled amyloid fibrils.Science advances · 2024Article
- Association between Parkinson's Disease and Cancer: New Findings and Possible Mediators.International journal of molecular sciences · 2024Review
74 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
8 authors at 2 institutions in 2 countries.
Funding
Abstract
Phenylketonuria (PKU) is characterized by phenylalanine accumulation and progressive mental retardation caused by an unknown mechanism. We demonstrate that at pathological concentrations, phenylalanine self-assembles into fibrils with amyloid-like morphology and well-ordered electron diffraction. These assemblies are specifically recognized by antibodies, show cytotoxicity that can be neutralized by the antibodies and are present in the hippocampus of model mice and in parietal cortex brain tissue from individuals with PKU. This is, to our knowledge, the first demonstration that a single amino acid can form amyloid-like deposits, suggesting a new amyloidosis-like etiology for PKU.
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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.