ArticleActa neuropathologica communications2013
Deciphering the pathogenesis of sporadic Creutzfeldt-Jakob disease with codon 129 M/V and type 2 abnormal prion protein.
Article in Acta neuropathologica communications, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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27 citing papers in PubMed, 72 citations in OpenAlex.
- Plaque-type dura mater graft-associated Creutzfeldt-Jakob disease: an autopsied case report.Prion · 2026Article
- The Phenotypic Spectrum of Sporadic Creutzfeldt-Jakob Disease Cortical Subtype.Annals of neurology · 2026Article
- Unsuccessful transmissions of atypical genetic Creutzfeldt-Jakob disease (PRNP p.T183A-129M) in transgenic mice.Acta neuropathologica · 2024Article
- Transmission experiments verify sporadic V2 prion in a patient with E200K mutation.Acta neuropathologica · 2024Article
- Novel histotypes of sporadic Creutzfeldt-Jakob disease linked to 129MV genotype.Acta neuropathologica communications · 2023Article
- Defining the phenotypic spectrum of sporadic Creutzfeldt-Jakob disease MV2K: the kuru plaque type.Brain : a journal of neurology · 2023Article
- Heterozygosity for cervid S138N polymorphism results in subclinical CWD in gene-targeted mice and progressive inhibition of prion conversion.Proceedings of the National Academy of Sciences of the United States of America · 2023Article
- PMCA-Based Detection of Prions in the Olfactory Mucosa of Patients With Sporadic Creutzfeldt-Jakob Disease.Frontiers in aging neuroscience · 2022Article
- Article
- Sporadic Creutzfeldt-Jakob disease: Real-Time Quaking Induced Conversion (RT-QuIC) assay represents a major diagnostic advance.European journal of histochemistry : EJH · 2021Review
- Phenotypic diversity of genetic Creutzfeldt-Jakob disease: a histo-molecular-based classification.Acta neuropathologica · 2021Article
- Two distinct conformers of PrPActa neuropathologica communications · 2021Article
- A novel mechanism of phenotypic heterogeneity in Creutzfeldt-Jakob disease.Acta neuropathologica communications · 2020Article
- Transmission characteristics of heterozygous cases of Creutzfeldt-Jakob disease with variable abnormal prion protein allotypes.Acta neuropathologica communications · 2020Article
- Co-existence of PrPScientific reports · 2020Article
- Understanding Prion Strains: Evidence from Studies of the Disease Forms Affecting Humans.Viruses · 2019Review
- A domain responsible for spontaneous conversion of bank vole prion protein.Brain pathology (Zurich, Switzerland) · 2019Article
- Recent advances in the histo-molecular pathology of human prion disease.Brain pathology (Zurich, Switzerland) · 2019Review
- Co-occurrence of chronic traumatic encephalopathy and prion disease.Acta neuropathologica communications · 2018Article
- Strains of Pathological Protein Aggregates in Neurodegenerative Diseases.Discoveries (Craiova, Romania) · 2017Review
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8 authors at 5 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
backgroundSporadic Creutzfeldt-Jakob disease is classified according to the genotype at polymorphic codon 129 (M or V) of the prion protein (PrP) gene and the type (1 or 2) of abnormal isoform of PrP (PrPSc) in the brain. The most complicated entity in the current classification system is MV2, since it shows wide phenotypic variations, i.e., MV2 cortical form (MV2C), MV2 with kuru plaques (MV2K), or a mixed form (MV2K + C). To resolve their complicated pathogenesis, we performed a comprehensive analysis of the three MV2 subgroups based on histopathological, molecular, and transmission properties.
resultsIn histopathological and molecular analyses, MV2C showed close similarity to MM2 cortical form (MM2C) and could be easily discriminated from the other MV2 subgroups. By contrast, MV2K and MV2K + C showed the same molecular type and the same transmission type, and the sole difference between MV2K and MV2K + C was the presence of cortical pathology characteristic of MV2C/MM2C. The remarkable molecular feature of MV2K or MV2K + C was a mixture of type 2 PrPSc and intermediate type PrPSc, which shows intermediate electrophoretic mobility between types 1 and 2 PrPSc. Modeling experiments using PrP-humanized mice indicated that MV2K contains a mixture of intermediate type PrPSc with the 129M genotype (Mi PrPSc) and type 2 PrPSc with the 129V genotype (V2 PrPSc) that originated from V2 PrPSc, whereas MV2C + K may also contain type 2 PrPSc with the 129M genotype and cortical pathology (M2C PrPSc) that lacks infectivity to the PrP-humanized mice in addition to Mi and V2 PrPSc.
conclusionsTaken together, the present study suggests that the phenotypic heterogeneity of MV2 stems from their different PrPSc origin(s).
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