ArticleThe EMBO journal2001
Onset of ataxia and Purkinje cell loss in PrP null mice inversely correlated with Dpl level in brain.
Article in The EMBO journal, 2001. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 88 papers.
What it found
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Who cites it
88 citing papers in PubMed, 253 citations in OpenAlex.
- Prion Protein: The Molecule of Many Forms and Faces.International journal of molecular sciences · 2022Review
- Show Me Your Friends and I Tell You Who You Are: The Many Facets of Prion Protein in Stroke.Cells · 2020Review
- Review
- An ancient conserved role for prion protein in learning and memory.Biology open · 2018Article
- Conditional Deletion ofThe Journal of neuroscience : the official journal of the Society for Neuroscience · 2017Article
- The biological function of the cellular prion protein: an update.BMC biology · 2017Review
- Membrane-enriched proteome changes and prion protein expression during neural differentiation and in neuroblastoma cells.BMC genomics · 2017Article
- Prion disease: experimental models and reality.Acta neuropathologica · 2017Review
- Article
- Physiological Functions of the Cellular Prion Protein.Frontiers in molecular biosciences · 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
- Domain-Specific Activation of Death-Associated Intracellular Signalling Cascades by the Cellular Prion Protein in Neuroblastoma Cells.Molecular neurobiology · 2016Article
- Heat Shock Protein Beta-1 Modifies Anterior to Posterior Purkinje Cell Vulnerability in a Mouse Model of Niemann-Pick Type C Disease.PLoS genetics · 2016Article
- Cellular Prion Protein Promotes Neuronal Differentiation of Adipose-Derived Stem Cells by Upregulating miRNA-124.Journal of molecular neuroscience : MN · 2016Article
- Strictly co-isogenic C57BL/6J-Prnp-/- mice: A rigorous resource for prion science.The Journal of experimental medicine · 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
- A specific role for PRND in goat foetal Leydig cells is suggested by prion family gene expression during gonad development in goats and mice.FEBS open bio · 2016Article
- Transgenic over-expression of mammalian heparanase delays prion disease onset and progression.Biochemical and biophysical research communications · 2015Article
- Article
- Unaltered Prion Pathogenesis in a Mouse Model of High-Fat Diet-Induced Insulin Resistance.PloS one · 2015Article
28 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
7 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
PrP knockout mice in which only the open reading frame was disrupted ('Zürich I') remained healthy. However, more extensive deletions resulted in ataxia, Purkinje cell loss and ectopic expression in brain of Doppel (Dpl), encoded by the downstream gene, PRND: A new PrP knockout line, 'Zürich II', with a 2.9 kb PRNP: deletion, developed this phenotype at approximately 10 months (50% morbidity). A single PRNP: allele abolished the syndrome. Compound Zürich I/Zürich II heterozygotes had half the Dpl of Zürich II mice and developed symptoms 6 months later. Zürich II mice transgenic for a PRND:-containing cosmid expressed Dpl at twice the level and became ataxic approximately 5 months earlier. Thus, Dpl levels in brain and onset of the ataxic syndrome are inversely correlated.
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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.