ArticleThe Journal of neuroscience : the official journal of the Society for Neuroscience2005
Truncated prion protein and Doppel are myelinotoxic in the absence of oligodendrocytic PrPC.
Article in The Journal of neuroscience : the official journal of the Society for Neuroscience, 2005. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 46 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
46 citing papers in PubMed, 84 citations in OpenAlex.
- Roles of prion proteins in mammalian development.Animal cells and systems · 2024Review
- Oligodendrocyte death and myelin loss in the cuprizone model: an updated overview of the intrinsic and extrinsic causes of cuprizone demyelination.Molecular neurodegeneration · 2022Review
- Prion Protein: The Molecule of Many Forms and Faces.International journal of molecular sciences · 2022Review
- Harnessing the Physiological Functions of Cellular Prion Protein in the Kidneys: Applications for Treating Renal Diseases.Biomolecules · 2021Review
- Cellular Prion Protein (PrPc): Putative Interacting Partners and Consequences of the Interaction.International journal of molecular sciences · 2020Review
- Altered distribution, aggregation, and protease resistance of cellular prion protein following intracranial inoculation.PloS one · 2019Article
- Nogo receptor 1 regulates Caspr distribution at axo-glial units in the central nervous system.Scientific reports · 2017Article
- The biological function of the cellular prion protein: an update.BMC biology · 2017Review
- Prion disease: experimental models and reality.Acta neuropathologica · 2017Review
- Dual role of cellular prion protein in normal host and Alzheimer's disease.Proceedings of the Japan Academy. Series B, Physical and biological sciences · 2017Review
- Assessment of the PrPc Amino-Terminal Domain in Prion Species Barriers.Journal of virology · 2016Article
- Article
- 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
- Article
- White matter involvement in sporadic Creutzfeldt-Jakob disease.Brain : a journal of neurology · 2014Article
- Myelin damage due to local quantitative abnormalities in normal prion levels: evidence from subacute combined degeneration and multiple sclerosis.Journal of neurology · 2014Review
- Cellular prion protein and NMDA receptor modulation: protecting against excitotoxicity.Frontiers in cell and developmental biology · 2014Review
- The toxicity of antiprion antibodies is mediated by the flexible tail of the prion protein.Nature · 2013Article
- Conserved roles of the prion protein domains on subcellular localization and cell-cell adhesion.PloS one · 2013Article
- Prion protein regulates iron transport by functioning as a ferrireductase.Journal of Alzheimer's disease : JAD · 2013Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
8 authors at 2 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The cellular prion protein PrP(C) confers susceptibility to transmissible spongiform encephalopathies, yet its normal function is unknown. Although PrP(C)-deficient mice develop and live normally, expression of amino proximally truncated PrP(C) (DeltaPrP) or of its structural homolog Doppel (Dpl) causes cerebellar degeneration that is prevented by coexpression of full-length PrP(C). We now report that mice expressing DeltaPrP or Dpl suffer from widespread leukoencephalopathy. Oligodendrocyte-specific expression of full-length PrP(C) under control of the myelin basic protein (MBP) promoter repressed leukoencephalopathy and vastly extended survival but did not prevent cerebellar granule cell (CGC) degeneration. Conversely, neuron-specific PrP(C) expression under control of the neuron-specific enolase (NSE) promoter antagonized CGC degeneration but not leukoencephalopathy. PrP(C) was found in purified myelin and in cultured oligodendrocytes of both wild-type and MBP-PrP transgenic mice but not in NSE-PrP mice. These results identify white-matter damage as an extraneuronal PrP-associated pathology and suggest a previously unrecognized role of PrP(C) in myelin maintenance.
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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.