Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties

No abstract is available for this paper.

• Publication year

  2010

• Venue

  Proceedings of the National Academy of Sciences of the United States of America

• Publication date

  2010-06-14

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1073/pnas.1004688107PMID 20547859PMCID PMC2900653
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Low Density Subcellular Fractions Enhance Disease-specific Prion Protein Misfolding

  2010cited by this paper
• Transmissions of variant Creutzfeldt-Jakob disease from brain and lymphoreticular tissue show uniform and conserved bovine spongiform encephalopathy-related phenotypic properties on primary and secondary passage in wild-type mice.

  2009cited by this paper
• Beyond PrPres Type 1/Type 2 Dichotomy in Creutzfeldt-Jakob Disease

  2008cited by this paper
• Conversion of the BASE Prion Strain into the BSE Strain: The Origin of BSE?

  2007cited by this paper
• A General Model of Prion Strains and Their Pathogenicity

  2007cited by this paper
• Efficient Transmission and Characterization of Creutzfeldt–Jakob Disease Strains in Bank Voles

  2006cited by this paper
• Glycosylation of PrP and the transmissible spongiform encephalopathies species barrier

  2006cited by this paper
• Predicting susceptibility and incubation time of human-to-human transmission of vCJD.

  2006cited by this paper
• The presence of valine at residue 129 in human prion protein accelerates amyloid formation

  2005cited by this paper
• Human Prion Protein with Valine 129 Prevents Expression of Variant CJD Phenotype

  2004cited by this paper
• Methionine 129 Variant of Human Prion Protein Oligomerizes More Rapidly than the Valine 129 Variant

  2004cited by this paper
• Prion protein heterogeneity in sporadic but not variant Creutzfeldt–Jakob disease: U.K. cases 1991–2002

  2004cited by this paper
• Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies.

  2004cited by this paper
• Molecular classification of sporadic Creutzfeldt-Jakob disease.

  2003cited by this paper
• Distribution of codon 129 genotype in human growth hormone-treated CJD patients in France and the UK.

  2003cited by this paper
• Abbreviated incubation times for human prions in mice expressing a chimeric mouse–human prion protein transgene

  2003cited by this paper
• Humanized knock-in mice expressing chimeric prion protein showed varied susceptibility to different human prions.

  2003cited by this paper
• Prion susceptibility and protective alleles exhibit marked geographic differences

  2003cited by this paper
• Follicular dendritic cell of the knock-in mouse provides a new bioassay for human prions.

  2002cited by this paper
• BSE prions propagate as either variant CJD‐like or sporadic CJD‐like prion strains in transgenic mice expressing human prion protein

  2002influential reference
• Neuropathology of variant Creutzfeldt-Jakob disease.

  2002cited by this paper
• Genetic influence on the structural variations of the abnormal prion protein.

  2000cited by this paper
• Iatrogenic Creutzfeldt–Jakob disease at the millennium

  2000cited by this paper
• Classification of sporadic Creutzfeldt‐Jakob disease based on molecular and phenotypic analysis of 300 subjects

  1999cited by this paper
• Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease.

  1999influential reference
• Nobel Lecture: Prions

  1998cited by this paper
• Genotype at codon 129 and susceptibility to Creutzfeldt-Jakob disease.

  1998cited by this paper
• The same prion strain causes vCJD and BSE

  1997cited by this paper
• Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent

  1997cited by this paper
• Molecular basis of phenotypic variability in sporadc creudeldt‐jakob disease

  1996cited by this paper
• Human spongiform encephalopathy: The national institutes of health series of 300 cases of experimentally transmitted disease

  1994cited by this paper
• Mice devoid of PrP are resistant to scrapie.

  1993cited by this paper
• Neuropathology of spongiform encephalopathies in humans.

  1993cited by this paper
• Fatal familial insomnia and familial Creutzfeldt-Jakob disease: disease phenotype determined by a DNA polymorphism.

  1992cited by this paper
• Novel proteinaceous infectious particles cause scrapie.

  1982cited by this paper
• The sequential development of the brain lesion of scrapie in three strains of mice.

  1968cited by this paper

• Biological Fluid Biomarkers in Human Prion Diseases with a Note on Biosafety.

  2025cites this paper
• Characterization of prion strains and peripheral prion infectivity patterns in E200K genetic CJD patients

  2025cites this paper
• The Evolution of Experimental Rodent Models for Prion Diseases

  2025cites this paper
• Human PrP E219K: a new and promising substrate for robust RT-QuIC amplification of human prions with potential for strain discrimination

  2025cites this paper
• Distinct cerebrospinal fluid proteomic signatures define clinicopathological subtypes of sporadic Creutzfeldt-Jakob disease and predict patient survival

  2025cites this paper
• The Phenotypic Spectrum of Sporadic Creutzfeldt-Jakob Disease Cortical Subtype.

  2025cites this paper
• A Review of CAC-717, a Disinfectant Containing Calcium Hydrogen Carbonate Mesoscopic Crystals

  2025cites this paper
• Transmission and Characterization of Creutzfeldt–Jakob Disease and Chronic Wasting Disease in the North American Deer Mouse

  2025cites this paper
• Structures of Oligomeric States of Tau Protein, Amyloid-β, α-Synuclein and Prion Protein Implicated in Alzheimer’s Disease, Parkinson’s Disease and Prionopathies

  2024cites this paper
• Human PrP E219K as a new and promising substrate for RT-QuIC amplification of human prion strains: a first step towards strain discrimination

  2024cites this paper
• Creutzfeldt–Jakob disease and other prion diseases

  2024cites this paper
• “Prion-like” seeding and propagation of oligomeric protein assemblies in neurodegenerative disorders

  2024influential citation
• Sodium hypochlorite inactivation of human CJD prions

  2024cites this paper
• The Importance of Prion Research.

  2024cites this paper
• Unsuccessful transmissions of atypical genetic Creutzfeldt–Jakob disease (PRNP p.T183A-129M) in transgenic mice

  2024cites this paper
• Pathological spectrum of sporadic Creutzfeldt-Jakob disease.

  2024cites this paper
• Characterization of Laboratory-Confirmed Creutzfeldt-Jakob Disease From 3 Ontario Tertiary Care Centers Between 2012 and 2022: A Retrospective Cohort Study

  2024cites this paper
• New developments in prion disease research using genetically modified mouse models

  2023cites this paper
• Defining the phenotypic spectrum of sporadic Creutzfeldt–Jakob disease MV2K: the kuru plaque type

  2023cites this paper
• The smallest infectious substructure encoding the prion strain structural determinant revealed by spontaneous dissociation of misfolded prion protein assemblies

  2023cites this paper
• Human prion disease: molecular pathogenesis, and possible therapeutic targets and strategies

  2023cites this paper
• Sporadic Creutzfeldt–Jakob disease infected human cerebral organoids retain the original human brain subtype features following transmission to humanized transgenic mice

  2023cites this paper
• The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis

  2023cites this paper
• Novel histotypes of sporadic Creutzfeldt–Jakob disease linked to 129MV genotype

  2023cites this paper
• Sporadic Creutzfeldt-Jakob disease VM1: phenotypic and molecular characterization of a novel subtype of human prion disease

  2022cites this paper
• Populations of Tau Conformers Drive Prion-like Strain Effects in Alzheimer’s Disease and Related Dementias

  2022cites this paper
• Recent Advances in Prion Inactivation by Plasma Sterilizer

  2022cites this paper
• Proteins

  2022cites this paper
• Genetically engineered cellular models of prion propagation

  2022cites this paper
• Prion assemblies: structural heterogeneity, mechanisms of formation, and role in species barrier

  2022cites this paper
• Gene-Edited Cell Models to Study Chronic Wasting Disease

  2022cites this paper
• Genomic, transcriptomic and RNA editing analysis of human MM1 and VV2 sporadic Creutzfeldt-Jakob disease

  2022cites this paper
• Phenotypic diversity of genetic Creutzfeldt–Jakob disease: a histo-molecular-based classification

  2021cites this paper
• Genetic Creutzfeldt–Jakob disease‐M232R with the cooccurrence of multiple prion strains, M1 + M2C + M2T: Report of an autopsy case

  2021cites this paper
• Adaptive selection of a prion strain conformer corresponding to established North American CWD during propagation of novel emergent Norwegian strains in mice expressing elk or deer prion protein

  2021cites this paper
• Prion strains associated with iatrogenic CJD in French and UK human growth hormone recipients

  2021cites this paper
• Huntington’s disease: lessons from prion disorders

  2021cites this paper
• Two distinct conformers of PrPD type 1 of sporadic Creutzfeldt–Jakob disease with codon 129VV genotype faithfully propagate in vivo

  2021cites this paper
• PMCA-generated prions from the olfactory mucosa of patients with Fatal Familial Insomnia cause prion disease in mice

  2021cites this paper
• Potential for transmission of sporadic Creutzfeldt–Jakob disease through peripheral routes

  2021influential citation
• Generation of human chronic wasting disease in transgenic mice

  2021cites this paper
• Sensitive protein misfolding cyclic amplification of sporadic Creutzfeldt–Jakob disease prions is strongly seed and substrate dependent

  2021cites this paper
• Wide distribution of prion infectivity in the peripheral tissues of vCJD and sCJD patients

  2021cites this paper
• Diffusion-weighted magnetic resonance imaging in dura mater graft-associated Creutzfeldt-Jakob disease

  2020cites this paper
• A novel mechanism of phenotypic heterogeneity in Creutzfeldt-Jakob disease

  2020cites this paper
• Transmission characteristics of heterozygous cases of Creutzfeldt-Jakob disease with variable abnormal prion protein allotypes

  2020cites this paper
• Prions from Sporadic Creutzfeldt-Jakob Disease Patients Propagate as Strain Mixtures

  2020influential citation
• The utility of bank voles for studying prion disease.

  2020cites this paper
• Co-existence of PrPD types 1 and 2 in sporadic Creutzfeldt-Jakob disease of the VV subgroup: phenotypic and prion protein characteristics

  2020cites this paper
• Cellular models for discovering prion disease therapeutics: Progress and challenges

  2020cites this paper
• Interventions to reduce the risk of surgically transmitted Creutzfeldt-Jakob disease: a cost-effective modelling review.

  2020cites this paper
• Improving the Predictive Value of Prion Inactivation Validation Methods to Minimize the Risks of Iatrogenic Transmission With Medical Instruments

  2020cites this paper
• Transmission of CJD from nasal brushings but not spinal fluid or RT‐QuIC product

  2020cites this paper
• Exposure Risk of Chronic Wasting Disease in Humans

  2020cites this paper
• Effect of Non-Concentrated and Concentrated Vaporized Hydrogen Peroxide on Scrapie Prions

  2020cites this paper
• Met166‐Glu168 residues in human PrP β2‐α2 loop account for evolutionary resistance to prion infection

  2020cites this paper
• Markers of A1 astrocytes stratify to molecular sub-types in sporadic Creutzfeldt–Jakob disease brain

  2020cites this paper
• Primary structural differences at residue 226 of deer and elk PrP dictate selection of distinct CWD prion strains in gene-targeted mice

  2019cites this paper
• Prions: Current Progress in Advanced Research

  2019cites this paper
• Gerstmann-Sträussler-Scheinker disease revisited: accumulation of covalently-linked multimers of internal prion protein fragments

  2019cites this paper
• Epitope mapping of the protease resistant products of RT-QuIC does not allow the discrimination of sCJD subtypes

  2019cites this paper
• Towards understanding selective neuronal vulnerability: establishing an in-vitro model for strain selection

  2019cites this paper
• In vitro Modeling of Prion Strain Tropism

  2019cites this paper
• A Novel Combination of Prion Strain Co-Occurrence in Patients with Sporadic Creutzfeldt-Jakob Disease.

  2019cites this paper
• Understanding Prion Strains: Evidence from Studies of the Disease Forms Affecting Humans

  2019influential citation
• Animal prion diseases: the risks to human health

  2019cites this paper
• Inactivation Methods for Prions.

  2019cites this paper
• Molecular Characterization of the Danish Prion Diseases Cohort With Special Emphasis on Rare and Unique Cases.

  2019cites this paper
• The characterization of AD/PART co-pathology in CJD suggests independent pathogenic mechanisms and no cross-seeding between misfolded Aβ and prion proteins

  2019cites this paper
• Recent advances in the histo‐molecular pathology of human prion disease

  2019influential citation
• Evidence for sporadic Creutzfeldt-Jakob disease being an acquired disease

  2019cites this paper
• Development of a quick bioassay for the evaluation of transmission properties of acquired prion diseases.

  2018cites this paper
• Regional pattern of microgliosis in sporadic Creutzfeldt‐Jakob disease in relation to phenotypic variants and disease progression

  2018cites this paper
• A domain responsible for spontaneous conversion of bank vole prion protein

  2018cites this paper
• Experimental models of human prion diseases and prion strains.

  2018cites this paper
• The zoonotic potential of animal prion diseases.

  2018cites this paper
• Artificial strain of human prions created in vitro

  2018cites this paper
• Iatrogenic Creutzfeldt-Jakob disease.

  2018cites this paper
• Sporadic Creutzfeldt-Jakob disease.

  2018cites this paper
• Explorer Analysis of conformational stability of abnormal prion protein aggregates across the spectrum of Creutzfeldt-Jakob disease

  2018cites this paper
• Prion diseases.

  2017cites this paper
• High diagnostic value of second generation CSF RT-QuIC across the wide spectrum of CJD prions

  2017cites this paper
• Insights into Mechanisms of Transmission and Pathogenesis from Transgenic Mouse Models of Prion Diseases.

  2017cites this paper
• Human stem cell–derived astrocytes replicate human prions in a PRNP genotype–dependent manner

  2017cites this paper
• Infectivity in bone marrow from sporadic CJD patients

  2017cites this paper
• Types and Strains: Their Essential Role in Understanding Protein Aggregation in Neurodegenerative Diseases

  2017cites this paper
• Detection of prion seeding activity in the olfactory mucosa of patients with Fatal Familial Insomnia

  2017cites this paper
• Prion disease: experimental models and reality

  2017cites this paper
• Application of protein misfolding cyclic amplification for the rapid diagnosis of acquired Creutzfeldt-Jakob disease

  2017cites this paper
• Novel strain properties distinguishing sporadic prion diseases sharing prion protein genotype and prion type

  2017cites this paper
• Cervid Prion Protein Polymorphisms Modulate the Diversity of Chronic Wasting Disease Prion Strains

  2017cites this paper
• Prions

  2017cites this paper
• TITLE : PRION STRAIN CHARACTERIZATION OF A NOVEL 1 SUBTYPE OF CREUTZFELDT-JAKOB DISEASE 2 RUNNING TITLE : Novel CJD prion strain 3

  2017influential citation
• The Use of Transgenic and Knockout Mice in Prion Research

  2017cites this paper
• How will prion disease evolve

  2017cites this paper
• Physiopathologie des formes infectieuses de maladies à prions humaines : étude des formes iatrogènes secondaires à un traitement par l'hormone de croissance

  2017cites this paper
• Towards an early clinical diagnosis of sporadic CJD VV2 (ataxic type)

  2017cites this paper
• Applications of the real-time quaking-induced conversion assay in diagnosis, prion strain-typing, drug pre-screening and other amyloidopathies

  2017cites this paper
• Bioassays and Inactivation of Prions.

  2017cites this paper
• Strains of Pathological Protein Aggregates in Neurodegenerative Diseases

  2017cites this paper