Lethal recessive myelin toxicity of prion protein lacking its central domain

Baumann F, Tolnay M, Brabeck C, Pahnke J, Kloz U, Niemann H, Heikenwalder M, Rulicke T, Burkle A, Aguzzi A (2007)
EMBO J 26(2): 538-547.

Zeitschriftenaufsatz | Veröffentlicht | Englisch
 
Download
Es wurden keine Dateien hochgeladen. Nur Publikationsnachweis!
DOI
Autor*in
Baumann, F.; Tolnay, M.; Brabeck, C.; Pahnke, J.; Kloz, U.; Niemann, HartmutUniBi ; Heikenwalder, M.; Rulicke, T.; Burkle, A.; Aguzzi, A.
Einrichtung
Centrum für Biotechnologie > Institut für Biochemie und Biotechnik
Fakultät für Chemie > Biochemie IV
Centrum für Biotechnologie > Arbeitsgruppe H. Niemann
Stichworte
Transgenic; Models; Mice; Inbred DBA; Inbred C57BL; Recessive; Mice; Inbred C3H; Mice; Mice; Genes; Gene Deletion; Biological; Lethal; Genes; Animals; Mutant Proteins/physiology; Myelin Sheath/*metabolism; Protein Structure; Mice; PrPC Proteins/chemistry/*genetics/physiology; Tertiary; Survival Analysis
Erscheinungsjahr
2007
Zeitschriftentitel
EMBO J
Band
26
Ausgabe
2
Seite(n)
538-547
ISSN
0261-4189
eISSN
1460-2075
Page URI
https://pub.uni-bielefeld.de/record/1927887

Zitieren

Baumann F, Tolnay M, Brabeck C, et al. Lethal recessive myelin toxicity of prion protein lacking its central domain. EMBO J. 2007;26(2):538-547.
Baumann, F., Tolnay, M., Brabeck, C., Pahnke, J., Kloz, U., Niemann, H., Heikenwalder, M., et al. (2007). Lethal recessive myelin toxicity of prion protein lacking its central domain. EMBO J, 26(2), 538-547. https://doi.org/10.1038/sj.emboj.7601510
Baumann, F., Tolnay, M., Brabeck, C., Pahnke, J., Kloz, U., Niemann, Hartmut, Heikenwalder, M., Rulicke, T., Burkle, A., and Aguzzi, A. 2007. “Lethal recessive myelin toxicity of prion protein lacking its central domain”. EMBO J 26 (2): 538-547.
Baumann, F., Tolnay, M., Brabeck, C., Pahnke, J., Kloz, U., Niemann, H., Heikenwalder, M., Rulicke, T., Burkle, A., and Aguzzi, A. (2007). Lethal recessive myelin toxicity of prion protein lacking its central domain. EMBO J 26, 538-547.
Baumann, F., et al., 2007. Lethal recessive myelin toxicity of prion protein lacking its central domain. EMBO J, 26(2), p 538-547.
F. Baumann, et al., “Lethal recessive myelin toxicity of prion protein lacking its central domain”, EMBO J, vol. 26, 2007, pp. 538-547.
Baumann, F., Tolnay, M., Brabeck, C., Pahnke, J., Kloz, U., Niemann, H., Heikenwalder, M., Rulicke, T., Burkle, A., Aguzzi, A.: Lethal recessive myelin toxicity of prion protein lacking its central domain. EMBO J. 26, 538-547 (2007).
Baumann, F., Tolnay, M., Brabeck, C., Pahnke, J., Kloz, U., Niemann, Hartmut, Heikenwalder, M., Rulicke, T., Burkle, A., and Aguzzi, A. “Lethal recessive myelin toxicity of prion protein lacking its central domain”. EMBO J 26.2 (2007): 538-547.

134 Zitationen in Europe PMC

Daten bereitgestellt von Europe PubMed Central.

Altered distribution, aggregation, and protease resistance of cellular prion protein following intracranial inoculation.
Ward A, Hollister JR, Choi YP, Race B, Williams K, Shoup DW, Moore RA, Priola SA., PLoS One 14(7), 2019
PMID: 31291644
Cellular Prion Protein as a Receptor of Toxic Amyloid-β42 Oligomers Is Important for Alzheimer's Disease.
Zhang Y, Zhao Y, Zhang L, Yu W, Wang Y, Chang W., Front Cell Neurosci 13(), 2019
PMID: 31417361
Lysosomal Quality Control in Prion Diseases.
Majumder P, Chakrabarti O., Mol Neurobiol 55(3), 2018
PMID: 28421536
The function of the cellular prion protein in health and disease.
Watts JC, Bourkas MEC, Arshad H., Acta Neuropathol 135(2), 2018
PMID: 29151170
Structural and mechanistic aspects influencing the ADAM10-mediated shedding of the prion protein.
Linsenmeier L, Mohammadi B, Wetzel S, Puig B, Jackson WS, Hartmann A, Uchiyama K, Sakaguchi S, Endres K, Tatzelt J, Saftig P, Glatzel M, Altmeppen HC., Mol Neurodegener 13(1), 2018
PMID: 29625583
Dual role of cellular prion protein in normal host and Alzheimer's disease.
Onodera T., Proc Jpn Acad Ser B Phys Biol Sci 93(4), 2017
PMID: 28413194
Insights into Mechanisms of Transmission and Pathogenesis from Transgenic Mouse Models of Prion Diseases.
Moreno JA, Telling GC., Methods Mol Biol 1658(), 2017
PMID: 28861793
Exploration of the Main Sites for the Transformation of Normal Prion Protein (PrPC) into Pathogenic Prion Protein (PrPsc).
Liu XL, Feng XL, Wang GM, Gong BB, Ahmad W, Liu NN, Zhang YY, Yang L, Ren HL, Cui SS., J Vet Res 61(1), 2017
PMID: 29978050
Regulation of RhoA activity by the cellular prion protein.
Kim HJ, Choi HS, Park JH, Kim MJ, Lee HG, Petersen RB, Kim YS, Park JB, Choi EK., Cell Death Dis 8(3), 2017
PMID: 28300846
The biological function of the cellular prion protein: an update.
Wulf MA, Senatore A, Aguzzi A., BMC Biol 15(1), 2017
PMID: 28464931
The N-terminus of the prion protein is a toxic effector regulated by the C-terminus.
Wu B, McDonald AJ, Markham K, Rich CB, McHugh KP, Tatzelt J, Colby DW, Millhauser GL, Harris DA., Elife 6(), 2017
PMID: 28527237
Loss of prion protein induces a primed state of type I interferon-responsive genes.
Malachin G, Reiten MR, Salvesen Ø, Aanes H, Kamstra JH, Skovgaard K, Heegaard PMH, Ersdal C, Espenes A, Tranulis MA, Bakkebø MK., PLoS One 12(6), 2017
PMID: 28651013
Inhibition of group-I metabotropic glutamate receptors protects against prion toxicity.
Goniotaki D, Lakkaraju AKK, Shrivastava AN, Bakirci P, Sorce S, Senatore A, Marpakwar R, Hornemann S, Gasparini F, Triller A, Aguzzi A., PLoS Pathog 13(11), 2017
PMID: 29176838
Prion protein inhibits fast axonal transport through a mechanism involving casein kinase 2.
Zamponi E, Buratti F, Cataldi G, Caicedo HH, Song Y, Jungbauer LM, LaDu MJ, Bisbal M, Lorenzo A, Ma J, Helguera PR, Morfini GA, Brady ST, Pigino GF., PLoS One 12(12), 2017
PMID: 29261664
Domain-Specific Activation of Death-Associated Intracellular Signalling Cascades by the Cellular Prion Protein in Neuroblastoma Cells.
Vilches S, Vergara C, Nicolás O, Mata Á, Del Río JA, Gavín R., Mol Neurobiol 53(7), 2016
PMID: 26250617
Mutated but Not Deleted Ovine PrP(C) N-Terminal Polybasic Region Strongly Interferes with Prion Propagation in Transgenic Mice.
Khalifé M, Reine F, Paquet-Fifield S, Castille J, Herzog L, Vilotte M, Moudjou M, Moazami-Goudarzi K, Makhzami S, Passet B, Andréoletti O, Vilette D, Laude H, Béringue V, Vilotte JL., J Virol 90(3), 2016
PMID: 26608316
Expression of the Prion Protein Family Member Shadoo Causes Drug Hypersensitivity That Is Diminished by the Coexpression of the Wild Type Prion Protein.
Nyeste A, Bencsura P, Vida I, Hegyi Z, Homolya L, Fodor E, Welker E., J Biol Chem 291(9), 2016
PMID: 26721882
A cationic tetrapyrrole inhibits toxic activities of the cellular prion protein.
Massignan T, Cimini S, Stincardini C, Cerovic M, Vanni I, Elezgarai SR, Moreno J, Stravalaci M, Negro A, Sangiovanni V, Restelli E, Riccardi G, Gobbi M, Castilla J, Borsello T, Nonno R, Biasini E., Sci Rep 6(), 2016
PMID: 26976106
Structural transitions in full-length human prion protein detected by xenon as probe and spin labeling of the N-terminal domain.
Narayanan SP, Nair DG, Schaal D, Barbosa de Aguiar M, Wenzel S, Kremer W, Schwarzinger S, Kalbitzer HR., Sci Rep 6(), 2016
PMID: 27341298
Generating Bona Fide Mammalian Prions with Internal Deletions.
Munoz-Montesino C, Sizun C, Moudjou M, Herzog L, Reine F, Chapuis J, Ciric D, Igel-Egalon A, Laude H, Béringue V, Rezaei H, Dron M., J Virol 90(15), 2016
PMID: 27226369
Identification of Anti-prion Compounds using a Novel Cellular Assay.
Imberdis T, Heeres JT, Yueh H, Fang C, Zhen J, Rich CB, Glicksman M, Beeler AB, Harris DA., J Biol Chem 291(50), 2016
PMID: 27803163
The prion protein family member Shadoo induces spontaneous ionic currents in cultured cells.
Nyeste A, Stincardini C, Bencsura P, Cerovic M, Biasini E, Welker E., Sci Rep 6(), 2016
PMID: 27819308
Decoding the function of the N-terminal tail of the cellular prion protein to inspire novel therapeutic avenues for neurodegenerative diseases.
Iraci N, Stincardini C, Barreca ML, Biasini E., Virus Res 207(), 2015
PMID: 25456402
The sheddase ADAM10 is a potent modulator of prion disease.
Altmeppen HC, Prox J, Krasemann S, Puig B, Kruszewski K, Dohler F, Bernreuther C, Hoxha A, Linsenmeier L, Sikorska B, Liberski PP, Bartsch U, Saftig P, Glatzel M., Elife 4(), 2015
PMID: 25654651
Neurodegeneration and unfolded-protein response in mice expressing a membrane-tethered flexible tail of PrP.
Dametto P, Lakkaraju AK, Bridel C, Villiger L, O'Connor T, Herrmann US, Pelczar P, Rülicke T, McHugh D, Adili A, Aguzzi A., PLoS One 10(2), 2015
PMID: 25658480
The elusive role of the prion protein and the mechanism of toxicity in prion disease.
Chiesa R., PLoS Pathog 11(5), 2015
PMID: 25951168
Involvement of PrP(C) in kainate-induced excitotoxicity in several mouse strains.
Carulla P, Llorens F, Matamoros-Angles A, Aguilar-Calvo P, Espinosa JC, Gavín R, Ferrer I, Legname G, Torres JM, del Río JA., Sci Rep 5(), 2015
PMID: 26155834
The Cellular Prion Protein: A Player in Immunological Quiescence.
Bakkebø MK, Mouillet-Richard S, Espenes A, Goldmann W, Tatzelt J, Tranulis MA., Front Immunol 6(), 2015
PMID: 26388873
The non-octarepeat copper binding site of the prion protein is a key regulator of prion conversion.
Giachin G, Mai PT, Tran TH, Salzano G, Benetti F, Migliorati V, Arcovito A, Della Longa S, Mancini G, D'Angelo P, Legname G., Sci Rep 5(), 2015
PMID: 26482532
Myelin damage due to local quantitative abnormalities in normal prion levels: evidence from subacute combined degeneration and multiple sclerosis.
Scalabrino G, Veber D., J Neurol 261(8), 2014
PMID: 24141733
A new paradigm for enzymatic control of α-cleavage and β-cleavage of the prion protein.
McDonald AJ, Dibble JP, Evans EG, Millhauser GL., J Biol Chem 289(2), 2014
PMID: 24247244
Interaction between 14-3-3β and PrP influences the dimerization of 14-3-3 and fibrillization of PrP106-126.
Han J, Song QQ, Sun P, Zhang J, Wang X, Song J, Li GQ, Liu YH, Mei GY, Shi Q, Tian C, Chen C, Gao C, Zhao B, Dong XP., Int J Biochem Cell Biol 47(), 2014
PMID: 24269782
Prion protein (PrP) gene-knockout cell lines: insight into functions of the PrP.
Sakudo A, Onodera T., Front Cell Dev Biol 2(), 2014
PMID: 25642423
PrP overdrive: does inhibition of α-cleavage contribute to PrP(C) toxicity and prion disease?
McDonald AJ, Millhauser GL., Prion 8(2), 2014
PMID: 24721836
Prion protein and cancers.
Yang X, Zhang Y, Zhang L, He T, Zhang J, Li C., Acta Biochim Biophys Sin (Shanghai) 46(6), 2014
PMID: 24681883
Evolutionary implications of metal binding features in different species' prion protein: an inorganic point of view.
La Mendola D, Rizzarelli E., Biomolecules 4(2), 2014
PMID: 24970230
Cellular prion protein and NMDA receptor modulation: protecting against excitotoxicity.
Black SA, Stys PK, Zamponi GW, Tsutsui S., Front Cell Dev Biol 2(), 2014
PMID: 25364752
A C-terminal membrane anchor affects the interactions of prion proteins with lipid membranes.
Chu NK, Shabbir W, Bove-Fenderson E, Araman C, Lemmens-Gruber R, Harris DA, Becker CF., J Biol Chem 289(43), 2014
PMID: 25217642
Relationships between cobalamin, epidermal growth factor, and normal prions in the myelin maintenance of central nervous system.
Scalabrino G, Veber D, Tredici G., Int J Biochem Cell Biol 55(), 2014
PMID: 25239885
Prions, prionoids and pathogenic proteins in Alzheimer disease.
Ashe KH, Aguzzi A., Prion 7(1), 2013
PMID: 23208281
Mammalian prions: tolerance to sequence changes-how far?
Salamat MK, Munoz-Montesino C, Moudjou M, Rezaei H, Laude H, Béringue V, Dron M., Prion 7(2), 2013
PMID: 23232499
Ataxia with cerebellar lesions in mice expressing chimeric PrP-Dpl protein.
Lemaire-Vieille C, Bailly Y, Erlich P, Loeuillet C, Brocard J, Haeberlé AM, Bombarde G, Rak C, Demais V, Dumestre-Pérard C, Gagnon J, Cesbron JY., J Neurosci 33(4), 2013
PMID: 23345215
Zinc drives a tertiary fold in the prion protein with familial disease mutation sites at the interface.
Spevacek AR, Evans EG, Miller JL, Meyer HC, Pelton JG, Millhauser GL., Structure 21(2), 2013
PMID: 23290724
Cobalamin and normal prions: a new horizon for cobalamin neurotrophism.
Scalabrino G, Veber D., Biochimie 95(5), 2013
PMID: 23328344
Roles of endoproteolytic α-cleavage and shedding of the prion protein in neurodegeneration.
Altmeppen HC, Prox J, Puig B, Dohler F, Falker C, Krasemann S, Glatzel M., FEBS J 280(18), 2013
PMID: 23413979
Prion disease: a tale of folds and strains.
Kretzschmar H, Tatzelt J., Brain Pathol 23(3), 2013
PMID: 23587138
Unique structural properties associated with mouse prion Δ105-125 protein.
Patel A, Vasiljevic S, Jones IM., Prion 7(3), 2013
PMID: 23764837
The toxicity of antiprion antibodies is mediated by the flexible tail of the prion protein.
Sonati T, Reimann RR, Falsig J, Baral PK, O'Connor T, Hornemann S, Yaganoglu S, Li B, Herrmann US, Wieland B, Swayampakula M, Rahman MH, Das D, Kav N, Riek R, Liberski PP, James MN, Aguzzi A., Nature 501(7465), 2013
PMID: 23903654
Conserved roles of the prion protein domains on subcellular localization and cell-cell adhesion.
Solis GP, Radon Y, Sempou E, Jechow K, Stuermer CA, Málaga-Trillo E., PLoS One 8(7), 2013
PMID: 23936187
Neurotoxicity of prion peptides mimicking the central domain of the cellular prion protein.
Vilches S, Vergara C, Nicolás O, Sanclimens G, Merino S, Varón S, Acosta GA, Albericio F, Royo M, Del Río JA, Gavín R., PLoS One 8(8), 2013
PMID: 23940658
Effects of FlAsH/tetracysteine (TC) Tag on PrP proteolysis and PrPres formation by TC-scanning.
Taguchi Y, Hohsfield LA, Hollister JR, Baron GS., Chembiochem 14(13), 2013
PMID: 23943295
Membrane-anchored Aβ accelerates amyloid formation and exacerbates amyloid-associated toxicity in mice.
Nagarathinam A, Höflinger P, Bühler A, Schäfer C, McGovern G, Jeffrey M, Staufenbiel M, Jucker M, Baumann F., J Neurosci 33(49), 2013
PMID: 24305824
Cobalamin (vitamin B(12)) regulation of PrP(C), PrP(C)-mRNA and copper levels in rat central nervous system.
Scalabrino G, Veber D, Mutti E, Calligaro A, Milani S, Tredici G., Exp Neurol 233(1), 2012
PMID: 22116041
PrPC interacts with potassium channel tetramerization domain containing 1 (KCTD1) protein through the PrP(51-136) region containing octapeptide repeats.
Huang T, Xu J, Xiang J, Lu Y, Chen R, Huang L, Xiao G, Sun G., Biochem Biophys Res Commun 417(1), 2012
PMID: 22138399
Prion protein at the crossroads of physiology and disease.
Biasini E, Turnbaugh JA, Unterberger U, Harris DA., Trends Neurosci 35(2), 2012
PMID: 22137337
Ion channels induced by the prion protein: mediators of neurotoxicity.
Solomon IH, Biasini E, Harris DA., Prion 6(1), 2012
PMID: 22453177
The toxicity of a mutant prion protein is cell-autonomous, and can be suppressed by wild-type prion protein on adjacent cells.
Biasini E, Turnbaugh JA, Massignan T, Veglianese P, Forloni G, Bonetto V, Chiesa R, Harris DA., PLoS One 7(3), 2012
PMID: 22428057
Prion potency in stem cells biology.
Lopes MH, Santos TG., Prion 6(2), 2012
PMID: 22437733
Integrity of helix 2-helix 3 domain of the PrP protein is not mandatory for prion replication.
Salamat K, Moudjou M, Chapuis J, Herzog L, Jaumain E, Béringue V, Rezaei H, Pastore A, Laude H, Dron M., J Biol Chem 287(23), 2012
PMID: 22511770
Five questions on prion diseases.
Aguzzi A, Zhu C., PLoS Pathog 8(5), 2012
PMID: 22570608
ZyFISH: a simple, rapid and reliable zygosity assay for transgenic mice.
McHugh D, O'Connor T, Bremer J, Aguzzi A., PLoS One 7(5), 2012
PMID: 22666404
Prion processing: a double-edged sword?
McMahon HE., Biochem Soc Trans 40(4), 2012
PMID: 22817725
Disease-associated mutations in the prion protein impair laminin-induced process outgrowth and survival.
Machado CF, Beraldo FH, Santos TG, Bourgeon D, Landemberger MC, Roffé M, Martins VR., J Biol Chem 287(52), 2012
PMID: 23132868
Cellular prion protein: from physiology to pathology.
Yusa S, Oliveira-Martins JB, Sugita-Konishi Y, Kikuchi Y., Viruses 4(11), 2012
PMID: 23202518
A Drug-Based Cellular Assay (DBCA) for studying cytotoxic and cytoprotective activities of the prion protein: A practical guide.
Massignan T, Biasini E, Harris DA., Methods 53(3), 2011
PMID: 21115124
Conserved stress-protective activity between prion protein and Shadoo.
Sakthivelu V, Seidel RP, Winklhofer KF, Tatzelt J., J Biol Chem 286(11), 2011
PMID: 21257747
An N-terminal polybasic domain and cell surface localization are required for mutant prion protein toxicity.
Solomon IH, Khatri N, Biasini E, Massignan T, Huettner JE, Harris DA., J Biol Chem 286(16), 2011
PMID: 21385869
Dissociation of infectivity from seeding ability in prions with alternate docking mechanism.
Miller MB, Geoghegan JC, Supattapone S., PLoS Pathog 7(7), 2011
PMID: 21779169
Proteasomal dysfunction and endoplasmic reticulum stress enhance trafficking of prion protein aggregates through the secretory pathway and increase accumulation of pathologic prion protein.
Nunziante M, Ackermann K, Dietrich K, Wolf H, Gädtke L, Gilch S, Vorberg I, Groschup M, Schätzl HM., J Biol Chem 286(39), 2011
PMID: 21835918
A nine amino acid domain is essential for mutant prion protein toxicity.
Westergard L, Turnbaugh JA, Harris DA., J Neurosci 31(39), 2011
PMID: 21957261
The N-terminal, polybasic region is critical for prion protein neuroprotective activity.
Turnbaugh JA, Westergard L, Unterberger U, Biasini E, Harris DA., PLoS One 6(9), 2011
PMID: 21980526
A naturally occurring C-terminal fragment of the prion protein (PrP) delays disease and acts as a dominant-negative inhibitor of PrPSc formation.
Westergard L, Turnbaugh JA, Harris DA., J Biol Chem 286(51), 2011
PMID: 22025612
Protease-resistant prions selectively decrease Shadoo protein.
Watts JC, Stöhr J, Bhardwaj S, Wille H, Oehler A, Dearmond SJ, Giles K, Prusiner SB., PLoS Pathog 7(11), 2011
PMID: 22163178
The octapeptide repeat PrP(C) region and cobalamin-deficient polyneuropathy of the rat.
Scalabrino G, Mutti E, Veber D, Rodriguez Menendez V, Novembrino C, Calligaro A, Tredici G., Muscle Nerve 44(6), 2011
PMID: 22102467
Prion protein: From physiology to cancer biology.
Mehrpour M, Codogno P., Cancer Lett 290(1), 2010
PMID: 19674833
Prion neurotoxicity: insights from prion protein mutants.
Solomon IH, Schepker JA, Harris DA., Curr Issues Mol Biol 12(2), 2010
PMID: 19767650
A novel, drug-based, cellular assay for the activity of neurotoxic mutants of the prion protein.
Massignan T, Stewart RS, Biasini E, Solomon IH, Bonetto V, Chiesa R, Harris DA., J Biol Chem 285(10), 2010
PMID: 19940127
Beta-amyloid oligomers and cellular prion protein in Alzheimer's disease.
Gunther EC, Strittmatter SM., J Mol Med (Berl) 88(4), 2010
PMID: 19960174
Axonal prion protein is required for peripheral myelin maintenance.
Bremer J, Baumann F, Tiberi C, Wessig C, Fischer H, Schwarz P, Steele AD, Toyka KV, Nave KA, Weis J, Aguzzi A., Nat Neurosci 13(3), 2010
PMID: 20098419
Unexpected tolerance of alpha-cleavage of the prion protein to sequence variations.
Oliveira-Martins JB, Yusa S, Calella AM, Bridel C, Baumann F, Dametto P, Aguzzi A., PLoS One 5(2), 2010
PMID: 20161712
Protein aggregation diseases: pathogenicity and therapeutic perspectives.
Aguzzi A, O'Connor T., Nat Rev Drug Discov 9(3), 2010
PMID: 20190788
Memory impairment in transgenic Alzheimer mice requires cellular prion protein.
Gimbel DA, Nygaard HB, Coffey EE, Gunther EC, Laurén J, Gimbel ZA, Strittmatter SM., J Neurosci 30(18), 2010
PMID: 20445063
A highly toxic cellular prion protein induces a novel, nonapoptotic form of neuronal death.
Christensen HM, Dikranian K, Li A, Baysac KC, Walls KC, Olney JW, Roth KA, Harris DA., Am J Pathol 176(6), 2010
PMID: 20472884
Neurotoxic mutants of the prion protein induce spontaneous ionic currents in cultured cells.
Solomon IH, Huettner JE, Harris DA., J Biol Chem 285(34), 2010
PMID: 20573963
Ionic mechanisms of action of prion protein fragment PrP(106-126) in rat basal forebrain neurons.
Alier K, Li Z, Mactavish D, Westaway D, Jhamandas JH., J Neurosci Res 88(10), 2010
PMID: 20175205
The hydrophobic core region governs mutant prion protein aggregation and intracellular retention.
Biasini E, Tapella L, Restelli E, Pozzoli M, Massignan T, Chiesa R., Biochem J 430(3), 2010
PMID: 20626348
The prion hypothesis: from biological anomaly to basic regulatory mechanism.
Tuite MF, Serio TR., Nat Rev Mol Cell Biol 11(12), 2010
PMID: 21081963
Review: contribution of transgenic models to understanding human prion disease.
Wadsworth JD, Asante EA, Collinge J., Neuropathol Appl Neurobiol 36(7), 2010
PMID: 20880036
Proteolysis of prion protein by cathepsin S generates a soluble beta-structured intermediate oligomeric form, with potential implications for neurotoxic mechanisms.
Polyakova O, Dear D, Stern I, Martin S, Hirst E, Bawumia S, Nash A, Dodson G, Bronstein I, Bayley PM., Eur Biophys J 38(2), 2009
PMID: 18813919
Perturbation of T-cell development by insertional mutation of a PrP transgene.
Zabel M, Greenwood C, Thackray AM, Pulford B, Rens W, Bujdoso R., Immunology 127(2), 2009
PMID: 19143847
A deleted prion protein that is neurotoxic in vivo is localized normally in cultured cells.
Christensen HM, Harris DA., J Neurochem 108(1), 2009
PMID: 19046329
De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis.
Sigurdson CJ, Nilsson KP, Hornemann S, Heikenwalder M, Manco G, Schwarz P, Ott D, Rülicke T, Liberski PP, Julius C, Falsig J, Stitz L, Wüthrich K, Aguzzi A., Proc Natl Acad Sci U S A 106(1), 2009
PMID: 19073920
Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers.
Laurén J, Gimbel DA, Nygaard HB, Gilbert JW, Strittmatter SM., Nature 457(7233), 2009
PMID: 19242475
The comprehensive native interactome of a fully functional tagged prion protein.
Rutishauser D, Mertz KD, Moos R, Brunner E, Rülicke T, Calella AM, Aguzzi A., PLoS One 4(2), 2009
PMID: 19209230
Fishing for prion protein function.
Chiesa R, Harris DA., PLoS Biol 7(3), 2009
PMID: 19338390
A systems approach to prion disease.
Hwang D, Lee IY, Yoo H, Gehlenborg N, Cho JH, Petritis B, Baxter D, Pitstick R, Young R, Spicer D, Price ND, Hohmann JG, Dearmond SJ, Carlson GA, Hood LE., Mol Syst Biol 5(), 2009
PMID: 19308092
Prion protein on astrocytes or in extracellular fluid impedes neurodegeneration induced by truncated prion protein.
Race B, Meade-White K, Race R, Baumann F, Aguzzi A, Chesebro B., Exp Neurol 217(2), 2009
PMID: 19332059
Early onset prion disease from octarepeat expansion correlates with copper binding properties.
Stevens DJ, Walter ED, Rodríguez A, Draper D, Davies P, Brown DR, Millhauser GL., PLoS Pathog 5(4), 2009
PMID: 19381258
Prion protein biosynthesis and its emerging role in neurodegeneration.
Chakrabarti O, Ashok A, Hegde RS., Trends Biochem Sci 34(6), 2009
PMID: 19447626
New insights into cellular prion protein (PrPc) functions: the "ying and yang" of a relevant protein.
Nicolas O, Gavín R, del Río JA., Brain Res Rev 61(2), 2009
PMID: 19523487
Reciprocal remodeling upon binding of the prion protein to its signaling partner hop/STI1.
Romano SA, Cordeiro Y, Lima LM, Lopes MH, Silva JL, Foguel D, Linden R., FASEB J 23(12), 2009
PMID: 19703931
Structural insights into alternate aggregated prion protein forms.
Polano M, Bek A, Benetti F, Lazzarino M, Legname G., J Mol Biol 393(5), 2009
PMID: 19720066
Functionally relevant domains of the prion protein identified in vivo.
Baumann F, Pahnke J, Radovanovic I, Rülicke T, Bremer J, Tolnay M, Aguzzi A., PLoS One 4(9), 2009
PMID: 19738901
The prion or the related Shadoo protein is required for early mouse embryogenesis.
Young R, Passet B, Vilotte M, Cribiu EP, Béringue V, Le Provost F, Laude H, Vilotte JL., FEBS Lett 583(19), 2009
PMID: 19766638
Prions: protein aggregation and infectious diseases.
Aguzzi A, Calella AM., Physiol Rev 89(4), 2009
PMID: 19789378
Is, indeed, the prion protein a Harlequin servant of "many" masters?
Sorgato MC, Peggion C, Bertoli A., Prion 3(4), 2009
PMID: 19887913
Role of prions in neuroprotection and neurodegeneration: a mechanism involving glutamate receptors?
Zamponi GW, Stys PK., Prion 3(4), 2009
PMID: 19684483
Context dependent neuroprotective properties of prion protein (PrP).
Steele AD, Zhou Z, Jackson WS, Zhu C, Auluck P, Moskowitz MA, Chesselet MF, Lindquist S., Prion 3(4), 2009
PMID: 19901559
De novo mammalian prion synthesis.
Benetti F, Legname G., Prion 3(4), 2009
PMID: 19887900
Cellular prion protein mediates the toxicity of beta-amyloid oligomers: implications for Alzheimer disease.
Nygaard HB, Strittmatter SM., Arch Neurol 66(11), 2009
PMID: 19901162
Doppel and PrPC co-immunoprecipitate in detergent-resistant membrane domains of epithelial FRT cells.
Caputo A, Sarnataro D, Campana V, Costanzo M, Negro A, Sorgato MC, Zurzolo C., Biochem J 425(2), 2009
PMID: 19888917
The deletion of amino acids 114-121 in the TM1 domain of mouse prion protein stabilizes its conformation but does not affect the overall structure.
Thaa B, Zahn R, Matthey U, Kroneck PM, Bürkle A, Fritz G., Biochim Biophys Acta 1783(6), 2008
PMID: 18088603
The two faces of protein misfolding: gain- and loss-of-function in neurodegenerative diseases.
Winklhofer KF, Tatzelt J, Haass C., EMBO J 27(2), 2008
PMID: 18216876
Molecular mechanisms of prion pathogenesis.
Aguzzi A, Sigurdson C, Heikenwaelder M., Annu Rev Pathol 3(), 2008
PMID: 18233951
The prion's elusive reason for being.
Aguzzi A, Baumann F, Bremer J., Annu Rev Neurosci 31(), 2008
PMID: 18558863
Physiology of the prion protein.
Linden R, Martins VR, Prado MA, Cammarota M, Izquierdo I, Brentani RR., Physiol Rev 88(2), 2008
PMID: 18391177
Retrotranslocation of prion proteins from the endoplasmic reticulum by preventing GPI signal transamidation.
Ashok A, Hegde RS., Mol Biol Cell 19(8), 2008
PMID: 18508914
Endocytosis of prion protein is required for ERK1/2 signaling induced by stress-inducible protein 1.
Caetano FA, Lopes MH, Hajj GN, Machado CF, Pinto Arantes C, Magalhães AC, Vieira Mde P, Américo TA, Massensini AR, Priola SA, Vorberg I, Gomez MV, Linden R, Prado VF, Martins VR, Prado MA., J Neurosci 28(26), 2008
PMID: 18579743
Dominant-negative effects of the N-terminal half of prion protein on neurotoxicity of prion protein-like protein/doppel in mice.
Yoshikawa D, Yamaguchi N, Ishibashi D, Yamanaka H, Okimura N, Yamaguchi Y, Mori T, Miyata H, Shigematsu K, Katamine S, Sakaguchi S., J Biol Chem 283(35), 2008
PMID: 18562311
Stress-protective signalling of prion protein is corrupted by scrapie prions.
Rambold AS, Müller V, Ron U, Ben-Tal N, Winklhofer KF, Tatzelt J., EMBO J 27(14), 2008
PMID: 18566584
Targeting prion proteins in neurodegenerative disease.
Gilch S, Krammer C, Schätzl HM., Expert Opin Biol Ther 8(7), 2008
PMID: 18549323
Antagonistic roles of the N-terminal domain of prion protein to doppel.
Sakaguchi S., Prion 2(3), 2008
PMID: 19158506
Green tea extracts interfere with the stress-protective activity of PrP and the formation of PrP.
Rambold AS, Miesbauer M, Olschewski D, Seidel R, Riemer C, Smale L, Brumm L, Levy M, Gazit E, Oesterhelt D, Baier M, Becker CF, Engelhard M, Winklhofer KF, Tatzelt J., J Neurochem 107(1), 2008
PMID: 18691383
Prion protein lacks robust cytoprotective activity in cultured cells.
Christensen HM, Harris DA., Mol Neurodegener 3(), 2008
PMID: 18718018
The octarepeat region of prion protein, but not the TM1 domain, is important for the antioxidant effect of prion protein.
Malaisé M, Schätzl HM, Bürkle A., Free Radic Biol Med 45(12), 2008
PMID: 18824094
The prion protein knockout mouse: a phenotype under challenge.
Steele AD, Lindquist S, Aguzzi A., Prion 1(2), 2007
PMID: 19164918
Bcl-2 overexpression delays caspase-3 activation and rescues cerebellar degeneration in prion-deficient mice that overexpress amino-terminally truncated prion.
Nicolas O, Gavín R, Braun N, Ureña JM, Fontana X, Soriano E, Aguzzi A, del Río JA., FASEB J 21(12), 2007
PMID: 17494993
Prion protein with an octapeptide insertion has impaired neuroprotective activity in transgenic mice.
Li A, Piccardo P, Barmada SJ, Ghetti B, Harris DA., EMBO J 26(11), 2007
PMID: 17510630
Doppel induces degeneration of cerebellar Purkinje cells independently of Bax.
Dong J, Li A, Yamaguchi N, Sakaguchi S, Harris DA., Am J Pathol 171(2), 2007
PMID: 17569776
Insights into prion strains and neurotoxicity.
Aguzzi A, Heikenwalder M, Polymenidou M., Nat Rev Mol Cell Biol 8(7), 2007
PMID: 17585315
In vitro and in vivo neurotoxicity of prion protein oligomers.
Simoneau S, Rezaei H, Salès N, Kaiser-Schulz G, Lefebvre-Roque M, Vidal C, Fournier JG, Comte J, Wopfner F, Grosclaude J, Schätzl H, Lasmézas CI., PLoS Pathog 3(8), 2007
PMID: 17784787
The CNS glycoprotein Shadoo has PrP(C)-like protective properties and displays reduced levels in prion infections.
Watts JC, Drisaldi B, Ng V, Yang J, Strome B, Horne P, Sy MS, Yoong L, Young R, Mastrangelo P, Bergeron C, Fraser PE, Carlson GA, Mount HT, Schmitt-Ulms G, Westaway D., EMBO J 26(17), 2007
PMID: 17703189
Prions and peripheral nerves: a deadly rendezvous.
Heikenwalder M, Julius C, Aguzzi A., J Neurosci Res 85(12), 2007
PMID: 17393538
Neurotoxic species in prion disease: a role for PrP isoforms?
Harrison CF, Barnham KJ, Hill AF., J Neurochem 103(5), 2007
PMID: 17944867

37 References

Daten bereitgestellt von Europe PubMed Central.

Mammalian prion biology: one century of evolving concepts.
Aguzzi A, Polymenidou M., Cell 116(2), 2004
PMID: 14744440
Nearly ubiquitous tissue distribution of the scrapie agent precursor protein.
Bendheim PE, Brown HR, Rudelli RD, Scala LJ, Goller NL, Wen GY, Kascsak RJ, Cashman NR, Bolton DC., Neurology 42(1), 1992
PMID: 1346470
A vector for expressing foreign genes in the brains and hearts of transgenic mice.
Borchelt DR, Davis J, Fischer M, Lee MK, Slunt HH, Ratovitsky T, Regard J, Copeland NG, Jenkins NA, Sisodia SS, Price DL., Genet. Anal. 13(6), 1996
PMID: 9117892
Prion protein protects human neurons against Bax-mediated apoptosis.
Bounhar Y, Zhang Y, Goodyer CG, LeBlanc A., J. Biol. Chem. 276(42), 2001
PMID: 11522774
Normal host prion protein necessary for scrapie-induced neurotoxicity.
Brandner S, Isenmann S, Raeber A, Fischer M, Sailer A, Kobayashi Y, Marino S, Weissmann C, Aguzzi A., Nature 379(6563), 1996
PMID: 8552188
Prion protein-deficient cells show altered response to oxidative stress due to decreased SOD-1 activity.
Brown DR, Schulz-Schaeffer WJ, Schmidt B, Kretzschmar HA., Exp. Neurol. 146(1), 1997
PMID: 9225743
Mice devoid of PrP are resistant to scrapie.
Bueler H, Aguzzi A, Sailer A, Greiner RA, Autenried P, Aguet M, Weissmann C., Cell 73(7), 1993
PMID: 8100741
Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein.
Bueler H, Fischer M, Lang Y, Bluethmann H, Lipp HP, DeArmond SJ, Prusiner SB, Aguet M, Weissmann C., Nature 356(6370), 1992
PMID: 1373228
Cellular prion protein transduces neuroprotective signals.
Chiarini LB, Freitas AR, Zanata SM, Brentani RR, Martins VR, Linden R., EMBO J. 21(13), 2002
PMID: 12093733
Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie.
Fischer M, Rulicke T, Raeber A, Sailer A, Moser M, Oesch B, Brandner S, Aguzzi A, Weissmann C., EMBO J. 15(6), 1996
PMID: 8635458

AUTHOR UNKNOWN, 2000
The role of PrP in health and disease.
Flechsig E, Weissmann C., Curr. Mol. Med. 4(4), 2004
PMID: 15354865
A transmembrane form of the prion protein in neurodegenerative disease.
Hegde RS, Mastrianni JA, Scott MR, DeFea KA, Tremblay P, Torchia M, DeArmond SJ, Prusiner SB, Lingappa VR., Science 279(5352), 1998
PMID: 9452375
Overexpression of nonconvertible PrPc delta114-121 in scrapie-infected mouse neuroblastoma cells leads to trans-dominant inhibition of wild-type PrP(Sc) accumulation.
Holscher C, Delius H, Burkle A., J. Virol. 72(2), 1998
PMID: 9445012
No superoxide dismutase activity of cellular prion protein in vivo.
Hutter G, Heppner FL, Aguzzi A., Biol. Chem. 384(9), 2003
PMID: 14515989
COOH-terminal sequence of the cellular prion protein directs subcellular trafficking and controls conversion into the scrapie isoform.
Kaneko K, Vey M, Scott M, Pilkuhn S, Cohen FE, Prusiner SB., Proc. Natl. Acad. Sci. U.S.A. 94(6), 1997
PMID: 9122195
Prions prevent neuronal cell-line death.
Kuwahara C, Takeuchi AM, Nishimura T, Haraguchi K, Kubosaki A, Matsumoto Y, Saeki K, Matsumoto Y, Yokoyama T, Itohara S, Onodera T., Nature 400(6741), 1999
PMID: 10421360
Neurotoxicity and neurodegeneration when PrP accumulates in the cytosol.
Ma J, Wollmann R, Lindquist S., Science 298(5599), 2002
PMID: 12386337
Prion protein is a component of the multimolecular signaling complex involved in T cell activation.
Mattei V, Garofalo T, Misasi R, Circella A, Manganelli V, Lucania G, Pavan A, Sorice M., FEBS Lett. 560(1-3), 2004
PMID: 14987990
Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel.
Moore RC, Lee IY, Silverman GL, Harrison PM, Strome R, Heinrich C, Karunaratne A, Pasternak SH, Chishti MA, Liang Y, Mastrangelo P, Wang K, Smit AF, Katamine S, Carlson GA, Cohen FE, Prusiner SB, Melton DW, Tremblay P, Hood LE, Westaway D., J. Mol. Biol. 292(4), 1999
PMID: 10525406
Signal transduction through prion protein.
Mouillet-Richard S, Ermonval M, Chebassier C, Laplanche JL, Lehmann S, Launay JM, Kellermann O., Science 289(5486), 2000
PMID: 10988071
Characterization of detergent-insoluble complexes containing the cellular prion protein and its scrapie isoform.
Naslavsky N, Stein R, Yanai A, Friedlander G, Taraboulos A., J. Biol. Chem. 272(10), 1997
PMID: 9045652
A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination.
Nishida N, Tremblay P, Sugimoto T, Shigematsu K, Shirabe S, Petromilli C, Erpel SP, Nakaoke R, Atarashi R, Houtani T, Torchia M, Sakaguchi S, DeArmond SJ, Prusiner SB, Katamine S., Lab. Invest. 79(6), 1999
PMID: 10378511
A cellular gene encodes scrapie PrP 27-30 protein.
Oesch B, Westaway D, Walchli M, McKinley MP, Kent SB, Aebersold R, Barry RA, Tempst P, Teplow DB, Hood LE., Cell 40(4), 1985
PMID: 2859120
Overexpression of PrPc triggers caspase 3 activation: potentiation by proteasome inhibitors and blockade by anti-PrP antibodies.
Paitel E, Alves da Costa C, Vilette D, Grassi J, Checler F., J. Neurochem. 83(5), 2002
PMID: 12437592
Cellular prion protein sensitizes neurons to apoptotic stimuli through Mdm2-regulated and p53-dependent caspase 3-like activation.
Paitel E, Fahraeus R, Checler F., J. Biol. Chem. 278(12), 2003
PMID: 12529324
Primary cultured neurons devoid of cellular prion display lower responsiveness to staurosporine through the control of p53 at both transcriptional and post-transcriptional levels.
Paitel E, Sunyach C, Alves da Costa C, Bourdon JC, Vincent B, Checler F., J. Biol. Chem. 279(1), 2003
PMID: 14570892
Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease.
Polymenidou M, Stoeck K, Glatzel M, Vey M, Bellon A, Aguzzi A., Lancet Neurol 4(12), 2005
PMID: 16297838
Novel proteinaceous infectious particles cause scrapie.
Prusiner SB., Science 216(4542), 1982
PMID: 6801762
Truncated prion protein and Doppel are myelinotoxic in the absence of oligodendrocytic PrPC.
Radovanovic I, Braun N, Giger OT, Mertz K, Miele G, Prinz M, Navarro B, Aguzzi A., J. Neurosci. 25(19), 2005
PMID: 15888663
The black cat/white cat principle of signal integration in bacterial promoters.
Cases I, de Lorenzo V., EMBO J. 20(1-2), 2001
PMID: 11226149
Pronuclear microinjection of mouse zygotes.
Rulicke T., Methods Mol. Biol. 254(), 2004
PMID: 15041762
Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions.
Shmerling D, Hegyi I, Fischer M, Blattler T, Brandner S, Gotz J, Rulicke T, Flechsig E, Cozzio A, von Mering C, Hangartner C, Aguzzi A, Weissmann C., Cell 93(2), 1998
PMID: 9568713
Cross-linking cellular prion protein triggers neuronal apoptosis in vivo.
Solforosi L, Criado JR, McGavern DB, Wirz S, Sanchez-Alavez M, Sugama S, DeGiorgio LA, Volpe BT, Wiseman E, Abalos G, Masliah E, Gilden D, Oldstone MB, Conti B, Williamson RA., Science 303(5663), 2004
PMID: 14752167
Perspectives: neurobiology. PrP's double causes trouble.
Weissmann C, Aguzzi A., Science 286(5441), 1999
PMID: 10577243
Three-dimensional structures of prion proteins.
Wuthrich K, Riek R., Adv. Protein Chem. 57(), 2001
PMID: 11447697
Stress-inducible protein 1 is a cell surface ligand for cellular prion that triggers neuroprotection.
Zanata SM, Lopes MH, Mercadante AF, Hajj GN, Chiarini LB, Nomizo R, Freitas AR, Cabral AL, Lee KS, Juliano MA, de Oliveira E, Jachieri SG, Burlingame A, Huang L, Linden R, Brentani RR, Martins VR., EMBO J. 21(13), 2002
PMID: 12093732
Export

Markieren/ Markierung löschen
Markierte Publikationen

Open Data PUB

Web of Science

Dieser Datensatz im Web of Science®
Quellen

PMID: 17245436
PubMed | Europe PMC

Suchen in

Google Scholar