Infecting human brain organoids with FFI or sCJD preserves prion traits regardless of host genotype
Prion diseases, such as sporadic Creutzfeldt-Jakob Disease (sCJD), are neurodegenerative disorders caused by misfolding of the prion protein (PrP). The D178N mutation in the PrP gene causes Fatal Familial Insomnia (FFI). Here we show that both sCJD and FFI prions can infect human cerebral organoids with or without the D178N mutation, and that the resulting infection is dictated by the inoculating prion and not the host organoid genotype.
Authors
- Bradley R. Groveman (ORCID: https://orcid.org/0000-0002-5059-7672)
- Cathryn L. Haigh (ORCID: https://orcid.org/0000-0001-7591-1149)
- Christina D. Orrú (ORCID: https://orcid.org/0000-0001-7253-2944)
- Brent Race (ORCID: https://orcid.org/0000-0002-5334-1023)
- Simote Totauhelotu Foliaki (ORCID: https://orcid.org/0000-0001-7450-6926)
- Gianluigi Zanusso (ORCID: https://orcid.org/0000-0001-5199-6264)
- Katie Williams (ORCID: https://orcid.org/0000-0002-7400-6056)
Institutions
- University of Verona (IT)
- National Institute of Allergy and Infectious Diseases (US)
Publication Details
- Journal
- npj Dementia
- Published
- 2025-09-16
- DOI
- https://doi.org/10.1038/s44400-025-00029-9
- Primary Topic
- Prion Diseases and Protein Misfolding
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- U.S. Department of Health and Human Services
- National Institutes of Health