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Open AccessArticle

Oral Ingestion of Synthetically Generated Recombinant Prion Is Sufficient to Cause Prion Disease in Wild-Type Mice

1
Key Laboratory of Brain Functional Genomics (Ministry of Education and Shanghai), Institute of Brain Functional Genomics, School of Life Sciences and the Collaborative Innovation Center for Brain Science, East China Normal University, Shanghai 200062, China
2
Fujian Provincial Key Laboratory for the Prevention and Control of Animal Infectious Diseases and Biotechnology, School of Life Sciences, Longyan University, Longyan 364012, China
3
Center for Neurodegenerative Science, Van Andel Institute, Grand Rapids, MI 49503, USA
*
Authors to whom correspondence should be addressed.
Pathogens 2020, 9(8), 653; https://doi.org/10.3390/pathogens9080653
Received: 22 July 2020 / Revised: 7 August 2020 / Accepted: 11 August 2020 / Published: 13 August 2020
Prion disease is a group of transmissible neurodegenerative disorders affecting humans and animals. The prion hypothesis postulates that PrPSc, the pathogenic conformer of host-encoded prion protein (PrP), is the unconventional proteinaceous infectious agent called prion. Supporting this hypothesis, highly infectious prion has been generated in vitro with recombinant PrP plus defined non-protein cofactors and the synthetically generated prion (recPrPSc) is capable of causing prion disease in wild-type mice through intracerebral (i.c.) or intraperitoneal (i.p.) inoculation. Given that many of the naturally occurring prion diseases are acquired through oral route, demonstrating the capability of recPrPSc to cause prion disease via oral transmission is important, but has never been proven. Here we showed for the first time that oral ingestion of recPrPSc is sufficient to cause prion disease in wild-type mice, which was supported by the development of fatal neurodegeneration in exposed mice, biochemical and histopathological analyses of diseased brains, and second round transmission. Our results demonstrate the oral transmissibility of recPrPSc and provide the missing evidence to support that the in vitro generated recPrPSc recapitulates all the important properties of naturally occurring prions. View Full-Text
Keywords: prion; recPrPSc; oral transmission; prion disease prion; recPrPSc; oral transmission; prion disease
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Pan, C.; Yang, J.; Zhang, X.; Chen, Y.; Wei, S.; Yu, G.; Pan, Y.-H.; Ma, J.; Yuan, C. Oral Ingestion of Synthetically Generated Recombinant Prion Is Sufficient to Cause Prion Disease in Wild-Type Mice. Pathogens 2020, 9, 653.

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