ArticleEmerging infectious diseases2013
Spontaneous generation of infectious prion disease in transgenic mice.
Article in Emerging infectious diseases, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 30 citations in OpenAlex.
- Substitution of histidine 95 by tyrosine in the prion protein causes spontaneous neurodegeneration in transgenic mice.PLoS pathogens · 2025Article
- Biological Fluid Biomarkers in Human Prion Diseases with a Note on Biosafety.Sub-cellular biochemistry · 2025Review
- Cell biology of prion strains in vivo and in vitro.Cell and tissue research · 2023Review
- Therapeutic Trial of anle138b in Mouse Models of Genetic Prion Disease.Journal of virology · 2023Article
- Bona fide atypical scrapie faithfully reproduced for the first time in a rodent model.Acta neuropathologica communications · 2022Article
- Homozygous R136S mutation in PRNP gene causes inherited early onset prion disease.Alzheimer's research & therapy · 2021Article
- Spontaneous generation of prions and transmissible PrP amyloid in a humanised transgenic mouse model of A117V GSS.PLoS biology · 2020Article
- Article
- Nonpathogenic Heterologous Prions Can Interfere with Prion Infection in a Strain-Dependent Manner.Journal of virology · 2018Article
- Experimental Models of Inherited PrP Prion Diseases.Cold Spring Harbor perspectives in medicine · 2017Review
- Generation of a new infectious recombinant prion: a model to understand Gerstmann-Sträussler-Scheinker syndrome.Scientific reports · 2017Article
- Review
- Towards authentic transgenic mouse models of heritable PrP prion diseases.Acta neuropathologica · 2016Article
- Transgenic fatal familial insomnia mice indicate prion infectivity-independent mechanisms of pathogenesis and phenotypic expression of disease.PLoS pathogens · 2015Article
- Mouse models for studying the formation and propagation of prions.The Journal of biological chemistry · 2014Review
Corrections and comments
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Authors and funding
11 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
We generated transgenic mice expressing bovine cellular prion protein (PrP(C)) 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 PrP(C) 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 PrP(C) sequence can induce spontaneous generation of an infectious prion disease that differs from all others identified in hosts expressing the same PrP(C) sequence. These observations support the view that a variety of infectious prion strains might spontaneously emerge in hosts displaying random genetic PrP(C) mutations.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.