Cerebral organoids as a new model for prion disease

Human brain has proven difficult to model, and this tissue is not widely available for study. [...]the application of organoid technology to produce 3D cultures of self-patterning brain tissue, human cerebral organoids (hCOs), offers great promise for investigating how brain cells become dysfunction...

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Bibliographic Details
Published in:PLoS pathogens 2021-07, Vol.17 (7), p.e1009747-e1009747
Main Authors: Groveman, Bradley R, Smith, Anna, Williams, Katie, Haigh, Cathryn L
Format: Article
Language:English
Subjects:
Animal models
Apoptosis
Asymptomatic
Bioaccumulation
Biochemistry
Biology and Life Sciences
Brain
Brain cells
Cell culture
Cell differentiation
Crosstalk
Cytokines
Disease
Endothelial cells
Health aspects
Infections
Inhibitory postsynaptic potentials
Investigations
Medical research
Medicine and Health Sciences
Medicine, Experimental
Metabolism
Methods
Microglia
Mutation
Neural stem cells
Neuroectoderm
Organoids
Organs, Culture of
Oxidative stress
Pattern formation
Pearls
Prion diseases
Prion protein
Prions
Proteins
Research and Analysis Methods
Stem cell transplantation
Stem cells
Technology
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Summary:Human brain has proven difficult to model, and this tissue is not widely available for study. [...]the application of organoid technology to produce 3D cultures of self-patterning brain tissue, human cerebral organoids (hCOs), offers great promise for investigating how brain cells become dysfunctional and die in neurodegenerative conditions. Organoids permit investigation into various aspects of prion disease including (1) different sCJD subtypes; (2) neuronal dysfunction and death; (3) cellular cross-talk; (4) conversion initiating triggers; and (5) efficacy of putative therapeutics. hCO, human cerebral organoid; PrP, prion protein; sCJD, sporadic Creutzfeldt–Jakob disease. https://doi.org/10.1371/journal.ppat.1009747.g001 Organoids become infected with and accumulate human prions Until 2017, no reproducible human cell model of prion infection existed. There was precedent in the literature from mouse models that neural stem cells (NSCs), and mature cultures differentiated from NSCs, would be permissible to infection [1–3]. [...]iPSCs were a prime technology to apply to this problem. Other cells are also involved in maintaining brain health, including microglia and endothelial cells, but these do not originate from the neuroectoderm lineage used to differentiate the neural lineage cells. [...]different approaches are being developed to study these cells.
ISSN:1553-7374
1553-7366
1553-7374
DOI:10.1371/journal.ppat.1009747