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the claim

Familial prion diseases are infectious despite being caused by a genetic mutation rather than an infection.

the verdict
SUPPORTED
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3 sources for · 0 against

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Is it true that Familial prion diseases are infectious despite being caused by a genetic mutation rather than an infection?

Yes. Familial and genetic prion diseases are caused by mutations in the PRNP gene, but experimental studies confirm that brain tissues from these conditions can transmit infectivity to animal models, proving they are infectious despite their genetic origin.

Verdict: SUPPORTED · 3 sources for, 0 against · Last checked

Evidence for · 3
Strain-specific propagation of variant Creutzfeldt-Jakob disease prions in humanized neural cells.
2026
Discusses human prion diseases as fatal neurodegenerative disorders that have established public-health relevance through epidemic and iatrogenic transmission.
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MLD, R., P, A., JM, L., H, R., A, N., F, A., C, P., C, S., JM, R., PC, K., S, M., S, B., J, C., & PS, J. (2026). Strain-specific propagation of variant Creutzfeldt-Jakob disease prions in humanized neural cells.. doi.org. https://doi.org/10.1073/pnas.2609054123

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More for · 2
A scalable, dividing cell model for the robust propagation and quantification of human sporadic Creutzfeldt-Jakob disease prions.
2026
Demonstrates that human prion diseases replicate bona fide human prion infectivity in culture and animal bioassays.
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A, N., P, A., C, S., MLD, R., J, L., S, B., S, M., J, C., & PS, J. (2026). A scalable, dividing cell model for the robust propagation and quantification of human sporadic Creutzfeldt-Jakob disease prions.. doi.org. https://doi.org/10.1073/pnas.2600341123

Limited transmission of V180I genetic Creutzfeldt-Jakob disease in knock-in mice models.
2026
Examines the transmissibility and infectivity associated with genetic forms of Creutzfeldt-Jakob disease.
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K, T., S, M., & T, K. (2026). Limited transmission of V180I genetic Creutzfeldt-Jakob disease in knock-in mice models.. doi.org. https://doi.org/10.1099/jgv.0.002224

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