ArticlePrion
PrP mRNA and protein expression in brain and PrP(c) in CSF in Creutzfeldt-Jakob disease MM1 and VV2.
Article in Prion. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 50 citations in OpenAlex.
- Exploring the Biological Connection Between Tau and PrPMolecular neurobiology · 2025Review
- PRNP expression predicts imaging findings in sporadic Creutzfeldt-Jakob disease.Annals of clinical and translational neurology · 2023Article
- Genomic, transcriptomic and RNA editing analysis of human MM1 and VV2 sporadic Creutzfeldt-Jakob disease.Acta neuropathologica communications · 2022Article
- The multiple functions of PrPJournal of molecular medicine (Berlin, Germany) · 2022Review
- Analysis of co-isogenic prion protein deficient mice reveals behavioral deficits, learning impairment, and enhanced hippocampal excitability.BMC biology · 2022Article
- Altered mRNA and Protein Expression of Monocarboxylate Transporter MCT1 in the Cerebral Cortex and Cerebellum of Prion Protein Knockout Mice.International journal of molecular sciences · 2021Article
- Regional Differences in Neuroinflammation-Associated Gene Expression in the Brain of Sporadic Creutzfeldt-Jakob Disease Patients.International journal of molecular sciences · 2020Article
- Tau Protein as a New Regulator of Cellular Prion Protein Transcription.Molecular neurobiology · 2020Article
- MicroRNA Alterations in a Tg501 Mouse Model of Prion Disease.Biomolecules · 2020Article
- Article
- Review
- RNA editing alterations define manifestation of prion diseases.Proceedings of the National Academy of Sciences of the United States of America · 2019Article
- Analysis of RNA Expression Profiles Identifies Dysregulated Vesicle Trafficking Pathways in Creutzfeldt-Jakob Disease.Molecular neurobiology · 2019Article
- Prion protein quantification in human cerebrospinal fluid as a tool for prion disease drug development.Proceedings of the National Academy of Sciences of the United States of America · 2019Article
- Cerebrospinal Fluid Total Prion Protein in the Spectrum of Prion Diseases.Molecular neurobiology · 2019Article
- Cytoskeleton-Associated Risk Modifiers Involved in Early and Rapid Progression of Sporadic Creutzfeldt-Jakob Disease.Molecular neurobiology · 2018Article
- Sisyphus in Neverland.Journal of Alzheimer's disease : JAD · 2018Article
- Regional and subtype-dependent miRNA signatures in sporadic Creutzfeldt-Jakob disease are accompanied by alterations in miRNA silencing machinery and biogenesis.PLoS pathogens · 2018Article
- Assessment of Chronic Wasting Disease Prion Shedding in Deer Saliva with Occupancy Modeling.Journal of clinical microbiology · 2018Article
- Molecular Alterations in the Cerebellum of Sporadic Creutzfeldt-Jakob Disease Subtypes with DJ-1 as a Key Regulator of Oxidative Stress.Molecular neurobiology · 2018Article
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Authors and funding
14 authors at 10 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Creutzfeldt-Jakob disease (CJD) is a heterogenic neurodegenerative disorder associated with abnormal post-translational processing of cellular prion protein (PrP(c)). CJD displays distinctive clinical and pathological features which correlate with the genotype at the codon 129 (methionine or valine: M or V respectively) in the prion protein gene and with size of the protease-resistant core of the abnormal prion protein PrP(sc) (type 1: 20/21 kDa and type 2: 19 kDa). MM1 and VV2 are the most common sporadic CJD (sCJD) subtypes. PrP mRNA expression levels in the frontal cortex and cerebellum are reduced in sCJD in a form subtype-dependent. Total PrP protein levels and PrP(sc) levels in the frontal cortex and cerebellum accumulate differentially in sCJD MM1 and sCJD VV2 with no relation between PrP(sc) deposition and spongiform degeneration and neuron loss, but with microgliosis, and IL6 and TNF-α response. In the CSF, reduced PrP(c), the only form present in this compartment, occurs in sCJD MM1 and VV2. PrP mRNA expression is also reduced in the frontal cortex in advanced stages of Alzheimer disease, Lewy body disease, progressive supranuclear palsy, and frontotemporal lobe degeneration, but PrP(c) levels in brain varies from one disease to another. Reduced PrP(c) levels in CSF correlate with PrP mRNA expression in brain, which in turn reflects severity of degeneration in sCJD.
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