B R Groveman, S T Foliaki, K Williams, C D Orrù, B Race, G Zanusso, C L Haigh
{"title":"无论宿主基因型如何，用FFI或sCJD感染人脑类器官均可保留朊病毒特征。","authors":"B R Groveman, S T Foliaki, K Williams, C D Orrù, B Race, G Zanusso, C L Haigh","doi":"10.1038/s44400-025-00029-9","DOIUrl":null,"url":null,"abstract":"<p><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>","PeriodicalId":520469,"journal":{"name":"NPJ dementia","volume":"1 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12493991/pdf/","citationCount":"0","resultStr":"{\"title\":\"Infecting human brain organoids with FFI or sCJD preserves prion traits regardless of host genotype.\",\"authors\":\"B R Groveman, S T Foliaki, K Williams, C D Orrù, B Race, G Zanusso, C L Haigh\",\"doi\":\"10.1038/s44400-025-00029-9\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><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>\",\"PeriodicalId\":520469,\"journal\":{\"name\":\"NPJ dementia\",\"volume\":\"1 1\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12493991/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"NPJ dementia\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1038/s44400-025-00029-9\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/9/16 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"NPJ dementia","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1038/s44400-025-00029-9","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/9/16 0:00:00","PubModel":"Epub","JCR":"","JCRName":"","Score":null,"Total":0}
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.
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