Spontaneous Generation of Infectious Prion Disease in Transgenic Mice
We generated transgenic mice expressing bovine cellular prion protein (PrPC) with a leucine substitution at codon 113 (113L). This protein is homologous to human protein with mutation 102L, and its genetic link with Gerstmann–Sträussler–Scheinker syndrome has been established. This mutation in bovin...
| Main Authors: | , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Centers for Disease Control and Prevention
2013-12-01
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| Series: | Emerging Infectious Diseases |
| Subjects: | |
| Online Access: | https://wwwnc.cdc.gov/eid/article/19/12/13-0106_article |
| _version_ | 1819275014195642368 |
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| author | Juan-María Torres Joaquín Castilla Belén Pintado Alfonso Gutiérrez-Adan Olivier Andréoletti Patricia Aguilar-Calvo Ana-Isabel Arroba Beatriz Parra-Arrondo Isidro Ferrer Jorge Manzanares Juan-Carlos Espinosa |
| author_facet | Juan-María Torres Joaquín Castilla Belén Pintado Alfonso Gutiérrez-Adan Olivier Andréoletti Patricia Aguilar-Calvo Ana-Isabel Arroba Beatriz Parra-Arrondo Isidro Ferrer Jorge Manzanares Juan-Carlos Espinosa |
| author_sort | Juan-María Torres |
| collection | DOAJ |
| description | We generated transgenic mice expressing bovine cellular prion protein (PrPC) with a leucine substitution at codon 113 (113L). This protein is homologous to human protein with mutation 102L, and its genetic link with Gerstmann–Sträussler–Scheinker syndrome has been established. This mutation in bovine PrPC causes a fully penetrant, lethal, spongiform encephalopathy. This genetic disease was transmitted by intracerebral inoculation of brain homogenate from ill mice expressing mutant bovine PrP to mice expressing wild-type bovine PrP, which indicated de novo generation of infectious prions. Our findings demonstrate that a single amino acid change in the PrPC sequence can induce spontaneous generation of an infectious prion disease that differs from all others identified in hosts expressing the same PrPC sequence. These observations support the view that a variety of infectious prion strains might spontaneously emerge in hosts displaying random genetic PrPC mutations. |
| first_indexed | 2024-12-23T23:17:35Z |
| format | Article |
| id | doaj.art-f4eec1200adf4068814944b3da7f0e6b |
| institution | Directory Open Access Journal |
| issn | 1080-6040 1080-6059 |
| language | English |
| last_indexed | 2024-12-23T23:17:35Z |
| publishDate | 2013-12-01 |
| publisher | Centers for Disease Control and Prevention |
| record_format | Article |
| series | Emerging Infectious Diseases |
| spelling | doaj.art-f4eec1200adf4068814944b3da7f0e6b2022-12-21T17:26:27ZengCenters for Disease Control and PreventionEmerging Infectious Diseases1080-60401080-60592013-12-0119121938194710.3201/eid1912.130106Spontaneous Generation of Infectious Prion Disease in Transgenic MiceJuan-María TorresJoaquín CastillaBelén PintadoAlfonso Gutiérrez-AdanOlivier AndréolettiPatricia Aguilar-CalvoAna-Isabel ArrobaBeatriz Parra-ArrondoIsidro FerrerJorge ManzanaresJuan-Carlos EspinosaWe generated transgenic mice expressing bovine cellular prion protein (PrPC) with a leucine substitution at codon 113 (113L). This protein is homologous to human protein with mutation 102L, and its genetic link with Gerstmann–Sträussler–Scheinker syndrome has been established. This mutation in bovine PrPC causes a fully penetrant, lethal, spongiform encephalopathy. This genetic disease was transmitted by intracerebral inoculation of brain homogenate from ill mice expressing mutant bovine PrP to mice expressing wild-type bovine PrP, which indicated de novo generation of infectious prions. Our findings demonstrate that a single amino acid change in the PrPC sequence can induce spontaneous generation of an infectious prion disease that differs from all others identified in hosts expressing the same PrPC sequence. These observations support the view that a variety of infectious prion strains might spontaneously emerge in hosts displaying random genetic PrPC mutations.https://wwwnc.cdc.gov/eid/article/19/12/13-0106_articleprions and related diseasesspontaneous generationtransmissible spongiform encephalitisTSEbovine spongiform encephalitisBSE |
| spellingShingle | Juan-María Torres Joaquín Castilla Belén Pintado Alfonso Gutiérrez-Adan Olivier Andréoletti Patricia Aguilar-Calvo Ana-Isabel Arroba Beatriz Parra-Arrondo Isidro Ferrer Jorge Manzanares Juan-Carlos Espinosa Spontaneous Generation of Infectious Prion Disease in Transgenic Mice Emerging Infectious Diseases prions and related diseases spontaneous generation transmissible spongiform encephalitis TSE bovine spongiform encephalitis BSE |
| title | Spontaneous Generation of Infectious Prion Disease in Transgenic Mice |
| title_full | Spontaneous Generation of Infectious Prion Disease in Transgenic Mice |
| title_fullStr | Spontaneous Generation of Infectious Prion Disease in Transgenic Mice |
| title_full_unstemmed | Spontaneous Generation of Infectious Prion Disease in Transgenic Mice |
| title_short | Spontaneous Generation of Infectious Prion Disease in Transgenic Mice |
| title_sort | spontaneous generation of infectious prion disease in transgenic mice |
| topic | prions and related diseases spontaneous generation transmissible spongiform encephalitis TSE bovine spongiform encephalitis BSE |
| url | https://wwwnc.cdc.gov/eid/article/19/12/13-0106_article |
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