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Stem Cell Models in Prion Research

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Prions and Diseases

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Abstract

Stem cells have the capacity to differentiate into the mature cells of any organ within the body. For this reason, they offer an interesting opportunity to model many cellular systems and their associated diseases. Prion diseases (PrDs) are a family of fatal neurodegenerative diseases caused by mis-folding of the prion protein (PrP), a protein lacking consensus on its native function. Stem cell models have been used for investigating PrP function and PrDs for over two decades. Within this time, the models and the understanding of their use have been substantially expanded. Herein, the utilizations of stem cell models and the contribution to knowledge that has emerged from their use are summarized.

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Acknowledgements

This work was funded by the Intramural Research Program of the NIH (NIAID). The author would like to thank Dr. James Carroll and Dr. Bradley Groveman for critical review of the manuscript. Figure illustrations were created with BioRender.com

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Authors and Affiliations

  1. Prion Cell Biology Unit, Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Hamilton, MT, USA

    Cathryn L. Haigh

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  1. Cathryn L. Haigh
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Correspondence to Cathryn L. Haigh .

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Editors and Affiliations

  1. Departments of Pathology and Neurology, National Prion Disease Pathology Surveillance Center, National Center for Regenerative Medicine Case Western Reserve University, School of Medicine, Cleveland, OH, USA

    Wen-Quan Zou

  2. Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA

    Pierluigi Gambetti

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Haigh, C.L. (2023). Stem Cell Models in Prion Research. In: Zou, WQ., Gambetti, P. (eds) Prions and Diseases. Springer, Cham. https://doi.org/10.1007/978-3-031-20565-1_16

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