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<p>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.</p>

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Infecting human brain organoids with FFI or sCJD preserves prion traits regardless of host genotype

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年9月16日

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  • B. R. Groveman,
  • S. T. Foliaki,
  • K. Williams,
  • C. D. Orrù,
  • B. Race,
  • G. Zanusso,
  • C. L. Haigh

摘要

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.