ReviewNeuropathology and applied neurobiology2010
Review: contribution of transgenic models to understanding human prion disease.
Review in Neuropathology and applied neurobiology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
41 citing papers in PubMed, 67 citations in OpenAlex.
- Multiomic analyses direct hypotheses for Creutzfeldt-Jakob disease risk genes.Brain : a journal of neurology · 2025Article
- Isolation of a novel human prion strain from a PRNP codon 129 heterozygous vCJD patient.PLoS pathogens · 2025Article
- Article
- A structural basis for prion strain diversity.Nature chemical biology · 2023Article
- Prion strains viewed through the lens of cryo-EM.Cell and tissue research · 2023Review
- Vaccines for prion diseases: a realistic goal?Cell and tissue research · 2023Review
- Overexpression of mouse prion protein in transgenic mice causes a non-transmissible spongiform encephalopathy.Scientific reports · 2022Article
- Humanized Transgenic Mice Are Resistant to Chronic Wasting Disease Prions From Norwegian Reindeer and Moose.The Journal of infectious diseases · 2022Article
- Chronic Wasting Disease (CWD) in Cervids and the Consequences of a Mutable Protein Conformation.ACS omega · 2022Review
- Newly Identified Deficiencies in the Multiple Sclerosis Central Nervous System and Their Impact on the Remyelination Failure.Biomedicines · 2022Review
- Neurotropic influenza A virus infection causes prion protein misfolding into infectious prions in neuroblastoma cells.Scientific reports · 2021Article
- Vaporized Hydrogen Peroxide and Ozone Gas Synergistically Reduce Prion Infectivity on Stainless Steel Wire.International journal of molecular sciences · 2021Article
- Strain-Dependent Prion Infection in Mice Expressing Prion Protein with Deletion of Central Residues 91-106.International journal of molecular sciences · 2020Article
- Structural effects of the highly protective V127 polymorphism on human prion protein.Communications biology · 2020Article
- Spontaneous generation of prions and transmissible PrP amyloid in a humanised transgenic mouse model of A117V GSS.PLoS biology · 2020Article
- Understanding Prion Strains: Evidence from Studies of the Disease Forms Affecting Humans.Viruses · 2019Review
- Recent Advances in Understanding Mammalian Prion Structure: A Mini Review.Frontiers in molecular neuroscience · 2019Article
- Evaluating the causality of novel sequence variants in the prion protein gene by example.Neurobiology of aging · 2018Article
- Experimental sheep BSE prions generate the vCJD phenotype when serially passaged in transgenic mice expressing human prion protein.Journal of the neurological sciences · 2018Article
- Prion Protein Devoid of the Octapeptide Repeat Region Delays Bovine Spongiform Encephalopathy Pathogenesis in Mice.Journal of virology · 2018Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
Transgenic mice expressing human prion protein in the absence of endogenous mouse prion protein faithfully replicate human prions. These models reproduce all of the key features of human disease, including long clinically silent incubation periods prior to fatal neurodegeneration with neuropathological phenotypes that mirror human prion strain diversity. Critical contributions to our understanding of human prion disease pathogenesis and aetiology have only been possible through the use of transgenic mice. These models have provided the basis for the conformational selection model of prion transmission barriers and have causally linked bovine spongiform encephalopathy with variant Creutzfeldt-Jakob disease. In the future these models will be essential for evaluating newly identified potentially zoonotic prion strains, for validating effective methods of prion decontamination and for developing effective therapeutic treatments for human prion disease.
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Registered trials
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.