Deletion of N-terminal residues 23-88 from prion protein (PrP) abrogates the potential to rescue PrP-deficient mice from PrP-like protein/doppel-induced Neurodegeneration

scientific article published on 19 May 2003

Deletion of N-terminal residues 23-88 from prion protein (PrP) abrogates the potential to rescue PrP-deficient mice from PrP-like protein/doppel-induced Neurodegeneration is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1074/JBC.M303655200
P698PubMed publication ID12759361

P50authorRyuichiro AtarashiQ88499298
P2093author name stringNoriyuki Nishida
Shigeru Katamine
Suehiro Sakaguchi
Kazuto Shigematsu
Shinji Goto
Takahito Kondo
P2860cites workNormal development and behaviour of mice lacking the neuronal cell-surface PrP proteinQ43522858
Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques.Q45962479
Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes.Q45970590
129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normalQ47562788
Prions prevent neuronal cell-line death.Q48152328
Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesionsQ48479400
Truncated forms of the human prion protein in normal brain and in prion diseasesQ48807964
Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic miceQ48963662
Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP geneQ49010616
Copper binding to the N-terminal tandem repeat regions of mammalian and avian prion protein.Q55065425
Brain copper content and cuproenzyme activity do not vary with prion protein expression levelQ73538276
Identification of a novel gene encoding a PrP-like protein expressed as chimeric transcripts fused to PrP exon 1/2 in ataxic mouse line with a disrupted PrP geneQ74126314
Prion Protein BiologyQ21972822
Stress-inducible protein 1 is a cell surface ligand for cellular prion that triggers neuroprotectionQ24534939
Doppel-induced cerebellar degeneration in transgenic miceQ24555049
Solution structure of the E200K variant of human prion protein. Implications for the mechanism of pathogenesis in familial prion diseasesQ27626645
Two different neurodegenerative diseases caused by proteins with similar structuresQ27630179
Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppelQ28145970
Mice devoid of PrP are resistant to scrapieQ28249108
Electron paramagnetic resonance evidence for binding of Cu(2+) to the C-terminal domain of the murine prion proteinQ28367705
The PrP-like protein Doppel binds copperQ28506863
Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injuryQ28511628
Copper stimulates endocytosis of the prion proteinQ28589040
Doppel is an N-glycosylated, glycosylphosphatidylinositol-anchored protein. Expression in testis and ectopic production in the brains of Prnp(0/0) mice predisposed to Purkinje cell lossQ30876363
In vivo conversion of cellular prion protein to pathogenic isoforms, as monitored by conformation-specific antibodiesQ31816626
Successful transmission of three mouse-adapted scrapie strains to murine neuroblastoma cell lines overexpressing wild-type mouse prion proteinQ33794942
Absence of the prion protein homologue Doppel causes male sterilityQ34079215
Small is not beautiful: antagonizing functions for the prion protein PrP(C) and its homologue Dpl.Q34536351
Peptide aggregation in neurodegenerative diseaseQ34738985
Conversion of truncated and elongated prion proteins into the scrapie isoform in cultured cellsQ36225344
Location and properties of metal-binding sites on the human prion protein.Q37104327
A scrapie-like unfolding intermediate of the prion protein domain PrP(121-231) induced by acidic pH.Q37376722
Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob diseaseQ39080950
Cellular prion protein transduces neuroprotective signalsQ39644937
Onset of ataxia and Purkinje cell loss in PrP null mice inversely correlated with Dpl level in brainQ39714572
Prion protein protects human neurons against Bax-mediated apoptosisQ40783610
Phorbol ester-regulated cleavage of normal prion protein in HEK293 human cells and murine neuronsQ40859808
Prion protein of 106 residues creates an artifical transmission barrier for prion replication in transgenic miceQ41618801
Physiological expression of the gene for PrP-like protein, PrPLP/Dpl, by brain endothelial cells and its ectopic expression in neurons of PrP-deficient mice ataxic due to Purkinje cell degenerationQ42048578
Prion protein expression and superoxide dismutase activityQ42991570
P433issue31
P407language of work or nameEnglishQ1860
P921main subjectneurodegenerationQ1755122
prion protein familyQ24724413
P304page(s)28944-28949
P577publication date2003-05-19
P1433published inJournal of Biological ChemistryQ867727
P1476titleDeletion of N-terminal residues 23-88 from prion protein (PrP) abrogates the potential to rescue PrP-deficient mice from PrP-like protein/doppel-induced Neurodegeneration
P478volume278

Reverse relations

cites work (P2860)
Q39486840A nine amino acid domain is essential for mutant prion protein toxicity
Q34673565Age-dependent impairment of eyeblink conditioning in prion protein-deficient mice.
Q37080562Antagonistic roles of the N-terminal domain of prion protein to doppel
Q36006619Cellular prion protein neuroprotective function: implications in prion diseases
Q36432753Cellular prion protein: from physiology to pathology
Q40451562Dominant-negative effects of the N-terminal half of prion protein on neurotoxicity of prion protein-like protein/doppel in mice
Q33501189Functionally relevant domains of the prion protein identified in vivo
Q40508891Genetic mapping of activity determinants within cellular prion proteins: N-terminal modules in PrPC offset pro-apoptotic activity of the Doppel helix B/B' region
Q37262632Helix 3 is necessary and sufficient for prion protein's anti-Bax function
Q34058767Instability of the octarepeat region of the human prion protein gene
Q35098319Male infertility and DNA damage in Doppel knockout and prion protein/Doppel double-knockout mice
Q34351433Mouse-hamster chimeric prion protein (PrP) devoid of N-terminal residues 23-88 restores susceptibility to 22L prions, but not to RML prions in PrP-knockout mice
Q28475611Prion protein paralog doppel protein interacts with alpha-2-macroglobulin: a plausible mechanism for doppel-mediated neurodegeneration
Q41970102Prion protein with an octapeptide insertion has impaired neuroprotective activity in transgenic mice
Q35023923Prion strain-dependent differences in conversion of mutant prion proteins in cell culture
Q26782895The Cellular Prion Protein: A Player in Immunological Quiescence
Q34043470The N-terminal, polybasic region is critical for prion protein neuroprotective activity
Q42151993The alpha-secretase-derived N-terminal product of cellular prion, N1, displays neuroprotective function in vitro and in vivo
Q36003019The cellular prion protein (PrP(C)): its physiological function and role in disease
Q41870342The prion protein and its paralogue Doppel affect calcium signaling in Chinese hamster ovary cells.
Q37080450The prion protein knockout mouse: a phenotype under challenge
Q42008958The role of Bax and caspase-3 in doppel-induced apoptosis of cerebellar granule cells.
Q37074594Transgene-driven expression of the Doppel protein in Purkinje cells causes Purkinje cell degeneration and motor impairment

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