ReviewCurrent issues in molecular biology2010
Prion neurotoxicity: insights from prion protein mutants.
Review in Current issues in molecular biology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 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.
The trial behind it
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Who cites it
28 citing papers in PubMed, 59 citations in OpenAlex.
- Newly Identified Deficiencies in the Multiple Sclerosis Central Nervous System and Their Impact on the Remyelination Failure.Biomedicines · 2022Review
- Drosophila models of prionopathies: insight into prion protein function, transmission, and neurotoxicity.Current opinion in genetics & development · 2017Review
- Integrated Organotypic Slice Cultures and RT-QuIC (OSCAR) Assay: Implications for Translational Discovery in Protein Misfolding Diseases.Scientific reports · 2017Article
- Substitutions of PrP N-terminal histidine residues modulate scrapie disease pathogenesis and incubation time in transgenic mice.PloS one · 2017Article
- Domain-Specific Activation of Death-Associated Intracellular Signalling Cascades by the Cellular Prion Protein in Neuroblastoma Cells.Molecular neurobiology · 2016Article
- The Pathogenic A116V Mutation Enhances Ion-Selective Channel Formation by Prion Protein in Membranes.Biophysical journal · 2016Article
- A critical appraisal of the pathogenic protein spread hypothesis of neurodegeneration.Nature reviews. Neuroscience · 2016Review
- A cationic tetrapyrrole inhibits toxic activities of the cellular prion protein.Scientific reports · 2016Article
- Expression of the Prion Protein Family Member Shadoo Causes Drug Hypersensitivity That Is Diminished by the Coexpression of the Wild Type Prion Protein.The Journal of biological chemistry · 2016Article
- Activation of zebrafish Src family kinases by the prion protein is an amyloid-β-sensitive signal that prevents the endocytosis and degradation of E-cadherin/β-catenin complexes in vivo.Molecular neurodegeneration · 2016Article
- The prion protein is critical for DNA repair and cell survival after genotoxic stress.Nucleic acids research · 2015Article
- Gingerol prevents prion protein-mediated neuronal toxicity by regulating HIF prolyl hydroxylase 2 and prion protein.International journal of molecular medicine · 2014Article
- Myelin damage due to local quantitative abnormalities in normal prion levels: evidence from subacute combined degeneration and multiple sclerosis.Journal of neurology · 2014Review
- Review
- Probing early misfolding events in prion protein mutants by NMR spectroscopy.Molecules (Basel, Switzerland) · 2013Review
- A mutant prion protein sensitizes neurons to glutamate-induced excitotoxicity.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2013Article
- Ataxia with cerebellar lesions in mice expressing chimeric PrP-Dpl protein.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2013Article
- From prion diseases to prion-like propagation mechanisms of neurodegenerative diseases.International journal of cell biology · 2013Review
- Article
- Prion protein misfolding, strains, and neurotoxicity: an update from studies on Mammalian prions.International journal of cell biology · 2013Review
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
The chemical nature of prions and the mechanism by which they propagate are now reasonably well understood. In contrast, much less is known about the identity of the toxic prion protein (PrP) species that are responsible for neuronal death, and the cellular pathways that these forms activate. In addition, the normal, physiological function of cellular PrP (PrP(C)) has remained mysterious, hampering efforts to determine whether loss or alteration of this function contributes to the disease phenotype. Considerable evidence now suggests that aggregation, toxicity, and infectivity are distinct properties of PrP that do no necessarily coincide. In this review, we will discuss several mutant forms of PrP that produce spontaneous neurodegeneration in humans and/or transgenic mice without the formation of infectious PrP(Sc). These include an octapeptide insertional mutation, point mutations that favor synthesis of transmembrane forms of PrP, and deletions encompassing the central domain whose neurotoxicity is antagonized by the presence of wild-type PrP. By isolating the neurotoxic effects of PrP from the formation of infectious prions, these mutants have provided important insights into possible pathogenic mechanisms. These studies suggest that prion neurotoxicity may involve subversion of a cytoprotective activity of PrP(C) via altered signaling events at the plasma membrane.
Indexed as
Identifiers
19767650PMC4821541W1559685724What OpenQuestion holds
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.