ArticlePloS one2009
Functionally relevant domains of the prion protein identified in vivo.
Article in PloS one, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
What it found
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
20 citing papers in PubMed, 27 citations in OpenAlex.
- Newly Identified Deficiencies in the Multiple Sclerosis Central Nervous System and Their Impact on the Remyelination Failure.Biomedicines · 2022Review
- Neurometals in the Pathogenesis of Prion Diseases.International journal of molecular sciences · 2021Review
- Prion protein inhibits fast axonal transport through a mechanism involving casein kinase 2.PloS one · 2017Article
- Domain-Specific Activation of Death-Associated Intracellular Signalling Cascades by the Cellular Prion Protein in Neuroblastoma Cells.Molecular neurobiology · 2016Article
- The Structural Architecture of an Infectious Mammalian Prion Using Electron Cryomicroscopy.PLoS pathogens · 2016Article
- Generating Bona Fide Mammalian Prions with Internal Deletions.Journal of virology · 2016Article
- Involvement of PrP(C) in kainate-induced excitotoxicity in several mouse strains.Scientific reports · 2015Article
- Myelin damage due to local quantitative abnormalities in normal prion levels: evidence from subacute combined degeneration and multiple sclerosis.Journal of neurology · 2014Review
- Membrane-anchored Aβ accelerates amyloid formation and exacerbates amyloid-associated toxicity in mice.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
- Conserved roles of the prion protein domains on subcellular localization and cell-cell adhesion.PloS one · 2013Article
- Disease-associated mutations in the prion protein impair laminin-induced process outgrowth and survival.The Journal of biological chemistry · 2012Article
- Cellular prion protein: from physiology to pathology.Viruses · 2012Review
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- An N-terminal polybasic domain and cell surface localization are required for mutant prion protein toxicity.The Journal of biological chemistry · 2011Article
- Automated detection of amyloid-β-related cortical and subcortical signal changes in a transgenic model of Alzheimer's disease using high-field MRI.Journal of Alzheimer's disease : JAD · 2011Article
- Neurotoxic mutants of the prion protein induce spontaneous ionic currents in cultured cells.The Journal of biological chemistry · 2010Article
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- The alpha-secretase-derived N-terminal product of cellular prion, N1, displays neuroprotective function in vitro and in vivo.The Journal of biological chemistry · 2009Article
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The prion consists essentially of PrP(Sc), a misfolded and aggregated conformer of the cellular protein PrP(C). Whereas PrP(C) deficient mice are clinically healthy, expression of PrP(C) variants lacking its central domain (PrP(DeltaCD)), or of the PrP-related protein Dpl, induces lethal neurodegenerative syndromes which are repressed by full-length PrP. Here we tested the structural basis of these syndromes by grafting the amino terminus of PrP(C) (residues 1-134), or its central domain (residues 90-134), onto Dpl. Further, we constructed a soluble variant of the neurotoxic PrP(DeltaCD) mutant that lacks its glycosyl phosphatidyl inositol (GPI) membrane anchor. Each of these modifications abrogated the pathogenicity of Dpl and PrP(DeltaCD) in transgenic mice. The PrP-Dpl chimeric molecules, but not anchorless PrP(DeltaCD), ameliorated the disease of mice expressing truncated PrP variants. We conclude that the amino proximal domain of PrP exerts a neurotrophic effect even when grafted onto a distantly related protein, and that GPI-linked membrane anchoring is necessary for both beneficial and deleterious effects of PrP and its variants.
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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.