ArticleThe Journal of neuroscience : the official journal of the Society for Neuroscience2007
N-terminally deleted forms of the prion protein activate both Bax-dependent and Bax-independent neurotoxic pathways.
Article in The Journal of neuroscience : the official journal of the Society for Neuroscience, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 46 citations in OpenAlex.
- Transgenic Overexpression of the Disordered Prion Protein N1 Fragment in Mice Does Not Protect Against Neurodegenerative Diseases Due to Impaired ER Translocation.Molecular neurobiology · 2020Article
- Altered distribution, aggregation, and protease resistance of cellular prion protein following intracranial inoculation.PloS one · 2019Article
- Cellular prion protein is present in mitochondria of healthy mice.Scientific reports · 2017Article
- Domain-Specific Activation of Death-Associated Intracellular Signalling Cascades by the Cellular Prion Protein in Neuroblastoma Cells.Molecular neurobiology · 2016Article
- Article
- Evolutionary implications of metal binding features in different species' prion protein: an inorganic point of view.Biomolecules · 2014Review
- Infection of prions and treatment of PrP106-126 alter the endogenous status of protein 14-3-3 and trigger the mitochondrial apoptosis possibly via activating Bax pathway.Molecular neurobiology · 2014Article
- Bax, Bcl2, and p53 differentially regulate neomycin- and gentamicin-induced hair cell death in the zebrafish lateral line.Journal of the Association for Research in Otolaryngology : JARO · 2013Article
- A mutant prion protein sensitizes neurons to glutamate-induced excitotoxicity.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2013Article
- Article
- Proteolytic processing of the prion protein in health and disease.American journal of neurodegenerative disease · 2012Article
- The extracellular regulated kinase-1 (ERK1) controls regulated alpha-secretase-mediated processing, promoter transactivation, and mRNA levels of the cellular prion protein.The Journal of biological chemistry · 2011Article
- A highly toxic cellular prion protein induces a novel, nonapoptotic form of neuronal death.The American journal of pathology · 2010Article
- A novel, drug-based, cellular assay for the activity of neurotoxic mutants of the prion protein.The Journal of biological chemistry · 2010Article
- Prion neurotoxicity: insights from prion protein mutants.Current issues in molecular biology · 2010Review
- 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
- Activation of p53-regulated pro-apoptotic signaling pathways in PrP-mediated myopathy.BMC genomics · 2009Article
- Prion protein lacks robust cytoprotective activity in cultured cells.Molecular neurodegeneration · 2008Article
- Diminishing apoptosis by deletion of Bax or overexpression of Bcl-2 does not protect against infectious prion toxicity in vivo.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2007Article
- Alteration of NF-kappaB activity leads to mitochondrial apoptosis after infection with pathological prion protein.Cellular microbiology · 2007Article
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
Abstract
Transgenic (Tg) mice expressing prion protein (PrP) with a deletion of the flexible, N-terminal tail encompassing residues 32-134 spontaneously develop ataxia, degeneration of cerebellar granule cells, and vacuolation of white matter in the brain and spinal cord, resulting in death by 3 months of age. These abnormalities are completely abrogated by coexpression of wild-type PrP from a single copy of the endogenous Prn-p gene. A similar but much more severe phenotype is seen in transgenic mice expressing PrP deleted for a conserved block of 21 amino acids (residues 105-125) within the N-terminal tail. The latter animals die within 1 week of birth in the absence of endogenous PrP, and fivefold overexpression of wild-type PrP is required to delay death beyond 1 year. To define the cellular pathways mediating the neurotoxicity of PrPdelta32-134 and PrPdelta105-125, we analyzed the effect of genetically deleting the proapoptotic protein Bax in mice expressing these neurotoxic forms of PrP. We find that Bax deletion in Tg(PrPdelta32-134) mice delays the development of clinical illness and slows apoptosis of cerebellar granule cells but has no effect on white matter degeneration. In contrast, Bax deletion has no effect on the clinical or neuropathological phenotype of Tg(delta105-125) mice. Our results indicate that Bax-related pathways mediate the initial neurotoxic actions of PrPdelta32-134 but that neurodegeneration induced by this protein as well as by PrPdelta105-125 also involves Bax-independent pathways.
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