Genetic mapping of activity determinants within cellular prion proteins: N-terminal modules in PrPC offset pro-apoptotic activity of the Doppel helix B/B' region

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Genetic mapping of activity determinants within cellular prion proteins: N-terminal modules in PrPC offset pro-apoptotic activity of the Doppel helix B/B' region is …
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scholarly articleQ13442814

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P356DOI10.1074/JBC.M404794200
P8608Fatcat IDrelease_yicuz4mmxbcizo25yywlkhi5yu
P698PubMed publication ID15459186

P50authorFrançois MajorQ28875656
P2093author name stringJing Yang
Peter Mastrangelo
Joel C Watts
Hans Kretzschmar
Man-Sun Sy
David Westaway
Paul E Fraser
Otto Windl
Melissa Marvi
Janaky Coomaraswamy
Bettina Drisaldi
Bob Strome
Howard T J Mount
Rosemary Ahrens
M Azhar Chishti
P2860cites workDoppel-induced cerebellar degeneration in transgenic miceQ24555049
PrionsQ24633319
Two different neurodegenerative diseases caused by proteins with similar structuresQ27630179
NMR structure of the human doppel proteinQ27640537
NMR structure of the mouse prion protein domain PrP(121-231)Q27733163
Solution structure of a 142-residue recombinant prion protein corresponding to the infectious fragment of the scrapie isoformQ27743024
Potential pseudoknots in the PrP-encoding mRNAQ28205609
Identification and inhibition of the ICE/CED-3 protease necessary for mammalian apoptosisQ28292299
Proteasomes and ubiquitin are involved in the turnover of the wild-type prion proteinQ28360418
The PrP-like protein Doppel binds copperQ28506863
Copper stimulates endocytosis of the prion proteinQ28589040
Thermodynamic prediction of conserved secondary structure: application to the RRE element of HIV, the tRNA-like element of CMV and the mRNA of prion proteinQ48064053
Prion protein is necessary for normal synaptic functionQ48087355
Prions prevent neuronal cell-line death.Q48152328
RNA molecules stimulate prion protein conversionQ48170423
Small, highly structured RNAs participate in the conversion of human recombinant PrP(Sen) to PrP(Res) in vitroQ48214478
Neurotoxicity of a prion protein fragmentQ48304223
Neurological illness in transgenic mice expressing a prion protein with an insertional mutation.Q48308927
Identification of cryptic nuclear localization signals in the prion proteinQ48350176
Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions.Q48373564
Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesionsQ48479400
Induction of HO-1 and NOS in doppel-expressing mice devoid of PrP: implications for doppel functionQ48907389
Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP geneQ49010616
Prion protein devoid of the octapeptide repeat region restores susceptibility to scrapie in PrP knockout mice.Q54037114
De novo generation of a PrPSc-like conformation in living cells.Q54071895
Copper binding to the N-terminal tandem repeat regions of mammalian and avian prion protein.Q55065425
Altered circadian activity rhythms and sleep in mice devoid of prion proteinQ59071240
Copper binding to the prion protein: structural implications of four identical cooperative binding sitesQ30558995
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
Copper(II)-induced conformational changes and protease resistance in recombinant and cellular PrP. Effect of protein age and deamidationQ30881188
Mapping Cu(II) binding sites in prion proteins by diethyl pyrocarbonate modification and matrix-assisted laser desorption ionization-time of flight (MALDI-TOF) mass spectrometric footprintingQ32031203
Normal neurogenesis and scrapie pathogenesis in neural grafts lacking the prion protein homologue DoppelQ33757335
Dominant-negative inhibition of prion formation diminished by deletion mutagenesis of the prion proteinQ33803953
Impairment of superoxide dismutase activation by N-terminally truncated prion protein (PrP) in PrP-deficient neuronal cell lineQ34221769
Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another proteinQ34294957
The prion gene complex encoding PrP(C) and Doppel: insights from mutational analysisQ34382758
Prion and doppel proteins bind to granule cells of the cerebellumQ34416093
Small is not beautiful: antagonizing functions for the prion protein PrP(C) and its homologue Dpl.Q34536351
Metal ions and prion diseasesQ34662087
Mammalian prion proteins: enigma, variation and vaccinationQ34691392
Structure and polymorphism of the mouse prion protein geneQ35573850
Mammalian prion biology: one century of evolving conceptsQ35639302
Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagationQ36581272
Accumulation of protease-resistant prion protein (PrP) and apoptosis of cerebellar granule cells in transgenic mice expressing a PrP insertional mutationQ36982880
Transgene-driven expression of the Doppel protein in Purkinje cells causes Purkinje cell degeneration and motor impairmentQ37074594
Molecular cloning and complete sequence of prion protein cDNA from mouse brain infected with the scrapie agentQ37396908
Mice deficient for prion protein exhibit normal neuronal excitability and synaptic transmission in the hippocampusQ37693769
Onset of ataxia and Purkinje cell loss in PrP null mice inversely correlated with Dpl level in brainQ39714572
Expression of truncated PrP targeted to Purkinje cells of PrP knockout mice causes Purkinje cell death and ataxiaQ39777445
The mechanism of internalization of glycosylphosphatidylinositol-anchored prion proteinQ39790846
Anterograde and retrograde intracellular trafficking of fluorescent cellular prion proteinQ40585724
Prion protein prevents human breast carcinoma cell line from tumor necrosis factor alpha-induced cell deathQ40595404
Intercellular transfer of the cellular prion proteinQ40700207
Prion protein protects human neurons against Bax-mediated apoptosisQ40783610
Caspase-8 is required for cell death induced by expanded polyglutamine repeats.Q40961806
Grand ideas floating freely. Conference on the new prion biology: basic science, diagnosis and therapyQ43129135
Normal development and behaviour of mice lacking the neuronal cell-surface PrP proteinQ43522858
Ablation of the metal ion-induced endocytosis of the prion protein by disease-associated mutation of the octarepeat regionQ43645286
Differences between the prion protein and its homolog Doppel: a partially structured state with implications for scrapie formationQ43897439
Essential role of the prion protein N terminus in subcellular trafficking and half-life of cellular prion protein.Q44102009
Neurotoxicity and neurodegeneration when PrP accumulates in the cytosolQ44184139
Mutant PrP is delayed in its exit from the endoplasmic reticulum, but neither wild-type nor mutant PrP undergoes retrotranslocation prior to proteasomal degradationQ44381518
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 NeurodegenerationQ44448848
Cytosolic prion protein is not toxic and protects against Bax-mediated cell death in human primary neuronsQ44548892
Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication.Q45979200
Release of the cellular prion protein from cultured cells after loss of its glycoinositol phospholipid anchorQ46096263
Identification of glycoinositol phospholipid linked and truncated forms of the scrapie prion proteinQ46251858
Prion protein selectively binds copper(II) ionsQ46492410
Scrapie prion protein contains a phosphatidylinositol glycolipidQ46814773
The N-terminal region of the prion protein ectodomain contains a lipid raft targeting determinantQ47757000
P433issue53
P407language of work or nameEnglishQ1860
P921main subjectapoptotic processQ14599311
prion protein familyQ24724413
P304page(s)55443-55454
P577publication date2004-09-29
P1433published inJournal of Biological ChemistryQ867727
P1476titleGenetic mapping of activity determinants within cellular prion proteins: N-terminal modules in PrPC offset pro-apoptotic activity of the Doppel helix B/B' region
P478volume279