ArticleThe EMBO journal2003
Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxia.
Article in The EMBO journal, 2003. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 79 citations in OpenAlex.
- 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
- Characterization of four new monoclonal antibodies against the distal N-terminal region of PrP(c).PeerJ · 2015Article
- Unaltered Prion Pathogenesis in a Mouse Model of High-Fat Diet-Induced Insulin Resistance.PloS one · 2015Article
- Role of stress-inducible protein-1 in recruitment of bone marrow derived cells into the ischemic brains.EMBO molecular medicine · 2013Article
- Prion disease: a tale of folds and strains.Brain pathology (Zurich, Switzerland) · 2013Review
- 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
- Article
- The toxicity of a mutant prion protein is cell-autonomous, and can be suppressed by wild-type prion protein on adjacent cells.PloS one · 2012Article
- Transient expressions of doppel and its structural analog prionDelta32-121 in SH-SY5Y cells caused cytotoxicity possibly by triggering similar apoptosis pathway.Molecular biology reports · 2010Article
- Autophagy and cell death of Purkinje cells overexpressing Doppel in Ngsk Prnp-deficient mice.Brain pathology (Zurich, Switzerland) · 2010Article
- Doppel and PrPC co-immunoprecipitate in detergent-resistant membrane domains of epithelial FRT cells.The Biochemical journal · 2009Article
- Activation of p53-regulated pro-apoptotic signaling pathways in PrP-mediated myopathy.BMC genomics · 2009Article
- Dominant-negative effects of the N-terminal half of prion protein on neurotoxicity of prion protein-like protein/doppel in mice.The Journal of biological chemistry · 2008Article
- The two faces of protein misfolding: gain- and loss-of-function in neurodegenerative diseases.The EMBO journal · 2008Review
- Fatal neurological disease in scrapie-infected mice induced for experimental autoimmune encephalomyelitis.Journal of virology · 2007Article
- Doppel induces degeneration of cerebellar Purkinje cells independently of Bax.The American journal of pathology · 2007Article
- N-terminally deleted forms of the prion protein activate both Bax-dependent and Bax-independent neurotoxic pathways.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2007Article
- Neonatal lethality in transgenic mice expressing prion protein with a deletion of residues 105-125.The EMBO journal · 2007Article
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Authors and funding
11 authors at 1 institution in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
PrP knockout mice with disruption of only the PrP-encoding region (Zürich I-type) remain healthy, whereas mice with deletions extending upstream of the PrP-encoding exon (Nagasaki-type) suffer Purkinje cell loss and ataxia, associated with ectopic expression of Doppel in brain, particularly in Purkinje cells. The phenotype is abrogated by co-expression of full-length PrP. Doppel is 25% similar to PrP, has the same globular fold, but lacks the flexible N-terminal tail. We now show that in Zürich I-type PrP-null mice, expression of N-terminally truncated PrP targeted to Purkinje cells also leads to Purkinje cell loss and ataxia, which are reversed by PrP. Doppel and truncated PrP probably cause Purkinje cell degeneration by the same mechanism.
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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.