ArticleScientific reports2021
Bank vole prion protein extends the use of RT-QuIC assays to detect prions in a range of inherited prion diseases.
Article in Scientific reports, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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Who cites it
24 citing papers in PubMed, 34 citations in OpenAlex.
- From a prion seed to a tree of discoveries: Byron Caughey's RT-QuIC legacy in protein misfolding research.Acta neuropathologica · 2026Review
- Leveraging the dominant-negative effect of the kuru-protective G127V prion protein variant as a novel therapeutic strategy.Neurobiology of disease · 2026Article
- Enhanced Sensitivity of a Modified Quaking-Induced Conversion Diagnostic Test for the Broad Detection of Sporadic and Inherited Prion Diseases: A Retrospective Study.Annals of neurology · 2026Article
- In vitro, cellular and in vivo studies of amyloid oligomers structure and toxicity: Challenges and advances.Protein science : a publication of the Protein Society · 2026Review
- Leveraging the dominant-negative effect of the kuru-protective G127V prion protein variant as a novel therapeutic strategy.bioRxiv : the preprint server for biology · 2026Article
- Advancing prion diagnostics: full-length human E200K RT-QuIC substrate facilitates prion detection in tear fluid and improves sensitivity in cerebrospinal fluid.Acta neuropathologica communications · 2026Article
- Genetic Creutzfeldt-Jakob disease linked to the E200K mutation: a large cohort study.Acta neuropathologica · 2026Article
- Recent advancements in QuIC-based diagnostic assays for sporadic and inherited prion diseases: focusing on the emerging role of ES-QuIC.Frontiers in neuroscience · 2026Review
- Detection of Human Prion Seeding Activity in Formalin-Fixed Paraffin-Embedded Archival Tissues.Neuropathology and applied neurobiology · 2025Article
- Prion Disease Diagnostic Biomarker Utility in Pre-symptomatic Disease.Sub-cellular biochemistry · 2025Review
- Advancing Prion Diagnostics: RT-QuIC Applications in Peripheral Tissues.Sub-cellular biochemistry · 2025Review
- Convergent generation of atypical prions in knockin mouse models of genetic prion disease.The Journal of clinical investigation · 2024Article
- Detection of prions in matching post-mortem skin and cerebrospinal fluid samples using second-generation real-time quaking-induced conversion assay.Scientific reports · 2024Article
- PMCA screening of retropharyngeal lymph nodes in white-tailed deer and comparisons with ELISA and IHC.Scientific reports · 2023Article
- Seed amplification and neurodegeneration marker trajectories in individuals at risk of prion disease.Brain : a journal of neurology · 2023Article
- RT-QuIC as ultrasensitive method for prion detection.Cell and tissue research · 2023Review
- Standardization of Data Analysis for RT-QuIC-Based Detection of Chronic Wasting Disease.Pathogens (Basel, Switzerland) · 2023Article
- Diagnostic performance of CSF biomarkers in a well-characterized Australian cohort of sporadic Creutzfeldt-Jakob disease.Frontiers in neurology · 2023Article
- Essential Components of Synthetic Infectious Prion Formation De Novo.Biomolecules · 2022Review
- A field-deployable diagnostic assay for the visual detection of misfolded prions.Scientific reports · 2022Article
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Authors and funding
8 authors at 2 institutions in 1 country.
Funding
Abstract
The cerebrospinal fluid (CSF) real-time quaking-induced conversion assay (RT-QuIC) is an ultrasensitive prion amyloid seeding assay for diagnosis of sporadic Creutzfeldt-Jakob disease (CJD) but several prion strains remain unexplored or resistant to conversion with commonly used recombinant prion protein (rPrP) substrates. Here, bank vole (BV) rPrP was used to study seeding by a wide range of archived post-mortem human CSF samples from cases of sporadic, acquired and various inherited prion diseases in high throughput 384-well format. BV rPrP substrate yielded positive reactions in 70/79 cases of sporadic CJD [Sensitivity 88.6% (95% CI 79.5-94.7%)], 1/2 variant CJD samples, and 9/20 samples from various inherited prion diseases; 5/57 non-prion disease control CSFs had positive reactions, yielding an overall specificity of 91.2% (95% CI 80.1-97.1%). Despite limitations of using post-mortem samples and our results' discrepancy with other studies, we demonstrated for the first time that BV rPrP is susceptible to conversion by human CSF samples containing certain prion strains not previously responsive in conventional rPrPs, thus justifying further optimisation for wider diagnostic and prognostic use.
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