ArticlePathogens (Basel, Switzerland)2020
Pathogenic Prion Protein Isoforms Are Not Present in Cerebral Organoids Generated from Asymptomatic Donors Carrying the E200K Mutation Associated with Familial Prion Disease.
Article in Pathogens (Basel, Switzerland), 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.
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Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it, 52 citations in OpenAlex.
- Mapping the Cerebral Organoid Landscape: A Systematic Review of Preclinical 3D Models in Neuroscience.Advanced healthcare materials · 2026Pooled it
- Proteasome mutations associated with CANDLE syndrome cause altered neuronal development by dysregulating polyamine synthesis.The Journal of clinical investigation · 2026Article
- PrPStem cell reports · 2026Article
- GPI-anchorless prion disease is sensitive to oxidative stress and shows potential for treatment with edaravone, based on iPS-derived neuron study.Cellular and molecular life sciences : CMLS · 2025Article
- Prion and "Prion-Like" Detection: From Conventional Methods to Microfluidics or Lab-on-Chip Platforms to Monitor Seeding and Spreading of Misfolded Proteins.Sub-cellular biochemistry · 2025Review
- Tracking Prions by RT-QuIC: An Update.Sub-cellular biochemistry · 2025Review
- Abnormal synaptic architecture in iPSC-derived neurons from a multi-generational family with genetic Creutzfeldt-Jakob disease.Stem cell reports · 2024Article
- Prion propagation and cellular dysfunction in prion disease: Disconnecting the dots.PLoS pathogens · 2023Article
- Mechanisms of prion-induced toxicity.Cell and tissue research · 2023Review
- Cell biology of prion strains in vivo and in vitro.Cell and tissue research · 2023Review
- Sporadic Creutzfeldt-Jakob disease infected human cerebral organoids retain the original human brain subtype features following transmission to humanized transgenic mice.Acta neuropathologica communications · 2023Article
- Altered energy metabolism in Fatal Familial Insomnia cerebral organoids is associated with astrogliosis and neuronal dysfunction.PLoS genetics · 2023Article
- Hereditary E200K mutation within the prion protein gene alters human iPSC derived cardiomyocyte function.Scientific reports · 2022Article
- Homodimeric complexes of the 90-231 human prion: a multilayered computational study based on FMO/GRID-DRY approach.Journal of molecular modeling · 2022Article
- 3D Human Organoids: The Next "Viral" Model for the Molecular Basis of Infectious Diseases.Biomedicines · 2022Review
- Stress and viral insults do not trigger E200K PrP conversion in human cerebral organoids.PloS one · 2022Article
- Neuronal excitatory-to-inhibitory balance is altered in cerebral organoid models of genetic neurological diseases.Molecular brain · 2021Article
- Cerebral organoids as a new model for prion disease.PLoS pathogens · 2021Review
- Cerebral organoids and their potential for studies of brain diseases in domestic animals.Veterinary research · 2021Review
- From Cell Culture to Organoids-Model Systems for Investigating Prion Strain Characteristics.Biomolecules · 2021Review
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8 authors at 2 institutions in 1 country.
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Abstract
Cerebral organoids (COs) are a self-organizing three-dimensional brain tissue mimicking the human cerebral cortex. COs are a promising new system for modelling pathological features of neurological disorders, including prion diseases. COs expressing normal prion protein (PrPC) are susceptible to prion infection when exposed to the disease isoforms of PrP (PrPD). This causes the COs to develop aspects of prion disease pathology considered hallmarks of disease, including the production of detergent-insoluble, protease-resistant misfolded PrPD species capable of seeding the production of more misfolded species. To determine whether COs can model aspects of familial prion diseases, we produced COs from donor fibroblasts carrying the E200K mutation, the most common cause of human familial prion disease. The mature E200K COs were assessed for the hallmarks of prion disease. We found that up to 12 months post-differentiation, E200K COs harbored no PrPD as confirmed by the absence of detergent-insoluble, protease-resistant, and seeding-active PrP species. Our results suggest that the presence of the E200K mutation within the prion gene is insufficient to cause disease in neuronal tissue. Therefore, other factors, such as further genetic modifiers or aging processes, may influence the onset of misfolding.
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