Lethal recessive myelin toxicity of prion protein lacking its central domain

scientific article

Lethal recessive myelin toxicity of prion protein lacking its central domain is …
instance of (P31):
scholarly articleQ13442814

External links are
P356DOI10.1038/SJ.EMBOJ.7601510
P932PMC publication ID1783444
P698PubMed publication ID17245436
P5875ResearchGate publication ID6558640

P50authorAdriano AguzziQ375410
Mathias HeikenwalderQ43369827
Markus TolnayQ47157920
Thomas RülickeQ57549501
Jens PahnkeQ59540336
P2093author name stringMarkus Tolnay
Hartmut H Niemann
Frank Baumann
Ulrich Kloz
Alexander Bürkle
Christine Brabeck
P2860cites workStress-inducible protein 1 is a cell surface ligand for cellular prion that triggers neuroprotectionQ24534939
Perspectives: neurobiology. PrP's double causes troubleQ28138888
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
NEUROBIOLOGY:PrP's Double Causes TroubleQ29544375
Novel proteinaceous infectious particles cause scrapieQ29547678
Overexpression of nonconvertible PrPc delta114-121 in scrapie-infected mouse neuroblastoma cells leads to trans-dominant inhibition of wild-type PrP(Sc) accumulationQ34069408
The black cat/white cat principle of signal integration in bacterial promotersQ34166888
Three-dimensional structures of prion proteinsQ34305023
A transmembrane form of the prion protein in neurodegenerative diseaseQ34454961
A cellular gene encodes scrapie PrP 27-30 proteinQ34557641
Mammalian prion biology: one century of evolving conceptsQ35639302
The role of PrP in health and diseaseQ35881598
COOH-terminal sequence of the cellular prion protein directs subcellular trafficking and controls conversion into the scrapie isoformQ36039920
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 is a component of the multimolecular signaling complex involved in T cell activation.Q40585109
Overexpression of PrPc triggers caspase 3 activation: potentiation by proteasome inhibitors and blockade by anti-PrP antibodiesQ40689387
Prion protein protects human neurons against Bax-mediated apoptosisQ40783610
Signal transduction through prion proteinQ40855079
Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie.Q41064177
Normal development and behaviour of mice lacking the neuronal cell-surface PrP proteinQ43522858
Neurotoxicity and neurodegeneration when PrP accumulates in the cytosolQ44184139
Cellular prion protein sensitizes neurons to apoptotic stimuli through Mdm2-regulated and p53-dependent caspase 3-like activationQ44279170
Primary cultured neurons devoid of cellular prion display lower responsiveness to staurosporine through the control of p53 at both transcriptional and post-transcriptional levelsQ44626756
Prions prevent neuronal cell-line death.Q48152328
A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelinationQ48180328
No superoxide dismutase activity of cellular prion protein in vivoQ48185616
Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesionsQ48479400
Nearly ubiquitous tissue distribution of the scrapie agent precursor proteinQ48595206
Prion protein-deficient cells show altered response to oxidative stress due to decreased SOD-1 activityQ48670908
Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob diseaseQ48691137
Characterization of detergent-insoluble complexes containing the cellular prion protein and its scrapie isoformQ48767220
Normal host prion protein necessary for scrapie-induced neurotoxicityQ49160879
A vector for expressing foreign genes in the brains and hearts of transgenic miceQ54965153
Truncated Prion Protein and Doppel Are Myelinotoxic in the Absence of Oligodendrocytic PrPCQ57083651
Cross-Linking Cellular Prion Protein Triggers Neuronal Apoptosis in VivoQ61463936
Pronuclear microinjection of mouse zygotesQ79820188
P433issue2
P407language of work or nameEnglishQ1860
P921main subjecttoxicityQ274160
prion protein familyQ24724413
P304page(s)538-547
P577publication date2007-01-01
P1433published inThe EMBO JournalQ1278554
P1476titleLethal recessive myelin toxicity of prion protein lacking its central domain
P478volume26

Reverse relations

cites work (P2860)
Q34397037A C-terminal membrane anchor affects the interactions of prion proteins with lipid membranes
Q41907406A Drug-Based Cellular Assay (DBCA) for studying cytotoxic and cytoprotective activities of the prion protein: A practical guide
Q36686430A cationic tetrapyrrole inhibits toxic activities of the cellular prion protein.
Q33882410A highly toxic cellular prion protein induces a novel, nonapoptotic form of neuronal death
Q35626193A naturally occurring C-terminal fragment of the prion protein (PrP) delays disease and acts as a dominant-negative inhibitor of PrPSc formation.
Q37459305A new paradigm for enzymatic control of α-cleavage and β-cleavage of the prion protein
Q39486840A nine amino acid domain is essential for mutant prion protein toxicity
Q33748230A novel, drug-based, cellular assay for the activity of neurotoxic mutants of the prion protein.
Q24656032A systems approach to prion disease
Q91771298Altered distribution, aggregation, and protease resistance of cellular prion protein following intracranial inoculation
Q34800606An N-terminal polybasic domain and cell surface localization are required for mutant prion protein toxicity
Q37080562Antagonistic roles of the N-terminal domain of prion protein to doppel
Q46611794Ataxia with cerebellar lesions in mice expressing chimeric PrP-Dpl protein
Q34094490Axonal prion protein is required for peripheral myelin maintenance
Q33757721Beta-amyloid oligomers and cellular prion protein in Alzheimer's disease
Q92649273Cellular Prion Protein as a Receptor of Toxic Amyloid-β42 Oligomers Is Important for Alzheimer's Disease
Q34391341Cellular prion protein and NMDA receptor modulation: protecting against excitotoxicity
Q22251089Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers
Q33768032Cellular prion protein mediates the toxicity of beta-amyloid oligomers: implications for Alzheimer disease
Q36432753Cellular prion protein: from physiology to pathology
Q57372680Characterization of the Properties and Trafficking of an Anchorless Form of the Prion Protein
Q34922583Conserved roles of the prion protein domains on subcellular localization and cell-cell adhesion
Q39604213Conserved stress-protective activity between prion protein and Shadoo
Q33595425Context dependent neuroprotective properties of prion protein (PrP).
Q30485527De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis
Q37627076De novo mammalian prion synthesis
Q38279734Decoding the function of the N-terminal tail of the cellular prion protein to inspire novel therapeutic avenues for neurodegenerative diseases
Q36481861Disease-associated mutations in the prion protein impair laminin-induced process outgrowth and survival
Q33967960Dissociation of infectivity from seeding ability in prions with alternate docking mechanism
Q40650405Domain-Specific Activation of Death-Associated Intracellular Signalling Cascades by the Cellular Prion Protein in Neuroblastoma Cells
Q40451562Dominant-negative effects of the N-terminal half of prion protein on neurotoxicity of prion protein-like protein/doppel in mice
Q33675107Doppel and PrPC co-immunoprecipitate in detergent-resistant membrane domains of epithelial FRT cells
Q35915982Doppel induces degeneration of cerebellar Purkinje cells independently of Bax
Q48379992Dramatic reduction of PrP C level and glycosylation in peripheral nerves following PrP knock-out from Schwann cells does not prevent transmissible spongiform encephalopathy neuroinvasion
Q39245173Dual role of cellular prion protein in normal host and Alzheimer's disease
Q28910222Early onset prion disease from octarepeat expansion correlates with copper binding properties
Q30352829Effects of FlAsH/tetracysteine (TC) Tag on PrP proteolysis and PrPres formation by TC-scanning.
Q37335792Endocytosis of prion protein is required for ERK1/2 signaling induced by stress-inducible protein 1.
Q33912630Evolutionary implications of metal binding features in different species' prion protein: an inorganic point of view
Q56763705Exploration of the Main Sites for the Transformation of Normal Prion Protein (PrP) into Pathogenic Prion Protein (PrP)
Q36744507Expression of the Prion Protein Family Member Shadoo Causes Drug Hypersensitivity That Is Diminished by the Coexpression of the Wild Type Prion Protein
Q33425420Fishing for prion protein function
Q34263032Five questions on prion diseases
Q33501189Functionally relevant domains of the prion protein identified in vivo
Q37093469Generating Bona Fide Mammalian Prions with Internal Deletions
Q38734225Identification of Anti-prion Compounds using a Novel Cellular Assay.
Q33296885In vitro and in vivo neurotoxicity of prion protein oligomers.
Q47099606Inhibition of group-I metabotropic glutamate receptors protects against prion toxicity.
Q40071080Insights into Mechanisms of Transmission and Pathogenesis from Transgenic Mouse Models of Prion Diseases
Q36856446Insights into prion strains and neurotoxicity
Q36003628Integrity of helix 2-helix 3 domain of the PrP protein is not mandatory for prion replication
Q27308826Involvement of PrP(C) in kainate-induced excitotoxicity in several mouse strains
Q35918928Ion channels induced by the prion protein: mediators of neurotoxicity.
Q37627093Is, indeed, the prion protein a Harlequin servant of "many" masters?
Q33834732Loss of prion protein induces a primed state of type I interferon-responsive genes.
Q39250023Lysosomal Quality Control in Prion Diseases
Q30424667Mammalian prions: tolerance to sequence changes-how far?
Q38800950Mechanisms of prion-induced neurodegeneration.
Q46963608Membrane-anchored Aβ accelerates amyloid formation and exacerbates amyloid-associated toxicity in mice.
Q30032301Memory impairment in transgenic Alzheimer mice requires cellular prion protein
Q36481434Mutated but Not Deleted Ovine PrP(C) N-Terminal Polybasic Region Strongly Interferes with Prion Propagation in Transgenic Mice
Q38154145Myelin damage due to local quantitative abnormalities in normal prion levels: evidence from subacute combined degeneration and multiple sclerosis
Q27316068Neurodegeneration and unfolded-protein response in mice expressing a membrane-tethered flexible tail of PrP
Q34074520Neurotoxic mutants of the prion protein induce spontaneous ionic currents in cultured cells
Q36974937Neurotoxic species in prion disease: a role for PrP isoforms?
Q28535083Neurotoxicity of prion peptides mimicking the central domain of the cellular prion protein
Q44706961Perturbation of T-cell development by insertional mutation of a PrP transgene.
Q34307236PrP overdrive: does inhibition of α-cleavage contribute to PrP(C) toxicity and prion disease?
Q38099068Prion disease: a tale of folds and strains
Q36767231Prion neurotoxicity: insights from prion protein mutants
Q37995916Prion potency in stem cells biology
Q38027947Prion processing: a double-edged sword?
Q38340595Prion protein (PrP) gene-knockout cell lines: insight into functions of the PrP.
Q38200475Prion protein and cancers
Q35740063Prion protein at the crossroads of physiology and disease.
Q35094535Prion protein biosynthesis and its emerging role in neurodegeneration
Q47271332Prion protein inhibits fast axonal transport through a mechanism involving casein kinase 2.
Q42745830Prion protein lacks robust cytoprotective activity in cultured cells
Q37290904Prion protein on astrocytes or in extracellular fluid impedes neurodegeneration induced by truncated prion protein
Q41970102Prion protein with an octapeptide insertion has impaired neuroprotective activity in transgenic mice
Q38064769Prions, prionoids and pathogenic proteins in Alzheimer disease
Q34098028Protease-resistant prions selectively decrease Shadoo protein
Q35311053Proteasomal dysfunction and endoplasmic reticulum stress enhance trafficking of prion protein aggregates through the secretory pathway and increase accumulation of pathologic prion protein
Q34101110Protein aggregation diseases: pathogenicity and therapeutic perspectives
Q46349859Proteolysis of prion protein by cathepsin S generates a soluble beta-structured intermediate oligomeric form, with potential implications for neurotoxic mechanisms
Q37745468Regulation of RhoA activity by the cellular prion protein
Q36796856Retrotranslocation of prion proteins from the endoplasmic reticulum by preventing GPI signal transamidation
Q34474868Review: contribution of transgenic models to understanding human prion disease.
Q89762189RhoA/ROCK Regulates Prion Pathogenesis by Controlling Connexin 43 Activity
Q37580683Role of prions in neuroprotection and neurodegeneration: a mechanism involving glutamate receptors?
Q38081918Roles of endoproteolytic α-cleavage and shedding of the prion protein in neurodegeneration.
Q39970242Stress-protective signalling of prion protein is corrupted by scrapie prions
Q92209952Structural Consequences of Copper Binding to the Prion Protein
Q52602016Structural and mechanistic aspects influencing the ADAM10-mediated shedding of the prion protein.
Q91800637Structural evidence for the critical role of the prion protein hydrophobic region in forming an infectious prion
Q37035847Structural transitions in full-length human prion protein detected by xenon as probe and spin labeling of the N-terminal domain
Q40093030The CNS glycoprotein Shadoo has PrP(C)-like protective properties and displays reduced levels in prion infections
Q26782895The Cellular Prion Protein: A Player in Immunological Quiescence
Q34043470The N-terminal, polybasic region is critical for prion protein neuroprotective activity
Q33793628The N-terminus of the prion protein is a toxic effector regulated by the C-terminus.
Q90227644The Role of Protein Misfolding and Tau Oligomers (TauOs) in Alzheimer's Disease (AD)
Q33623592The biological function of the cellular prion protein: an update
Q33408170The comprehensive native interactome of a fully functional tagged prion protein
Q35624321The elusive role of the prion protein and the mechanism of toxicity in prion disease.
Q39680946The hydrophobic core region governs mutant prion protein aggregation and intracellular retention
Q38826643The non-octarepeat copper binding site of the prion protein is a key regulator of prion conversion.
Q34412881The prion hypothesis: from biological anomaly to basic regulatory mechanism
Q37397364The prion protein family member Shadoo induces spontaneous ionic currents in cultured cells.
Q37080450The prion protein knockout mouse: a phenotype under challenge
Q35137829The sheddase ADAM10 is a potent modulator of prion disease.
Q34200990The toxicity of a mutant prion protein is cell-autonomous, and can be suppressed by wild-type prion protein on adjacent cells
Q27685314The toxicity of antiprion antibodies is mediated by the flexible tail of the prion protein
Q37064056The two faces of protein misfolding: gain- and loss-of-function in neurodegenerative diseases
Q33531737Unexpected tolerance of alpha-cleavage of the prion protein to sequence variations
Q39139955Unique structural properties associated with mouse prion Δ105-125 protein
Q36604907Zinc drives a tertiary fold in the prion protein with familial disease mutation sites at the interface
Q27302091ZyFISH: a simple, rapid and reliable zygosity assay for transgenic mice

Search more.