Insoluble cellular prion protein and its association with prion and Alzheimer diseases

References

• Knight RS, Will RG. Prion diseases. J Neurol Neurosurg Psychiatry 2004; 75:36 - 42
   PubMed Web of Science ®Google Scholar
• Hardy J, Selkoe DJ. The amyloid hypothesis of Alzheimer's disease: progress and problems on the road to therapeutics. Science 2002; 297:353 - 356
   PubMed Web of Science ®Google Scholar
• Prusiner SB. Prions. Proc Natl Acad Sci USA 1998; 95:13363 - 13383
   PubMed Web of Science ®Google Scholar
• Meyer RK, McKinley MP, Bowman KA, Braunfeld MB, Barry RA, Prusiner SB. Separation and properties of cellular and scrapie prion proteins. Proc Natl Acad Sci USA 1986; 83:2310 - 2314
   PubMed Web of Science ®Google Scholar
• Caughey BW, Dong A, Bhat KS, Ernst D, Hayes SF, Caughey WS. Secondary structure analysis of the scrapie-associated protein PrP 27–30 in water by infrared spectroscopy. Biochemistry 1991; 30:7672 - 7680
   PubMed Web of Science ®Google Scholar
• Pan KM, Baldwin M, Nguyen J, Gasset M, Serban A, Groth D, et al. Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins. Proc Natl Acad Sci USA 1993; 90:10962 - 10966
   PubMed Web of Science ®Google Scholar
• Bessen RA, Marsh RF. Identification of two biologically distinct strains of transmissible mink encephalopathy in hamsters. J Gen Virol 1992; 73:329 - 334
   PubMed Web of Science ®Google Scholar
• Parchi P, Castellani R, Capellari S, Ghetti B, Young K, Chen SG, et al. Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996; 39:767 - 778
   PubMed Web of Science ®Google Scholar
• Caughey B, Raymond GJ, Bessen RA. Strain-dependent differences in beta-sheet conformations of abnormal prion protein. J Biol Chem 1998; 273:32230 - 32235
   PubMed Web of Science ®Google Scholar
• Safar J, Wille H, Itri V, Groth D, Serban H, Torchia M, et al. Eight prion strains have PrP(Sc) molecules with different conformations. Nat Med 1998; 4:1157 - 1165
   PubMed Web of Science ®Google Scholar
• Zou WQ, Gambetti P. Prion: the chameleon protein. Cell Mol Life Sci 2007; 64:3266 - 3270
   PubMed Web of Science ®Google Scholar
• Collinge J, Clarke AR. A general model of prion strains and their pathogenicity. Science 2007; 318:930 - 936
   PubMed Web of Science ®Google Scholar
• Yuan J, Xiao X, McGeehan J, Dong Z, Cali I, Fujioka H, et al. Insoluble aggregates and protease-resistant conformers of prion protein in uninfected human brains. J Biol Chem 2006; 281:34848 - 34858
   PubMed Web of Science ®Google Scholar
• Linden R, Martins VR, Prado MA, Cammarota M, Izquierdo I, Brentani RR. Physiology of the prion protein. Physiol Rev 2008; 88:673 - 728
   PubMed Web of Science ®Google Scholar
• Weeber EJ, Levenson JM, Sweatt JD. Molecular genetics of human cognition. Mol Interv 2002; 2:376 - 391
   PubMedGoogle Scholar
• Salès N, Rodolfo K, Hässig R, Faucheux B, Di Giamberardino L, Moya KL. Cellular prion protein localization in rodent and primate brain. Eur J Neurosci 1998; 10:2464 - 2471
   PubMed Web of Science ®Google Scholar
• Fournier JG, Escaig-Haye F, Grigoriev V. Ultra structural localization of prion proteins: physiological and pathological implications. Microsc Res Tech 2000; 50:76 - 88
   PubMed Web of Science ®Google Scholar
• Herms J, Tings T, Gall S, Madlung A, Giese A, Siebert H, et al. Evidence of presynaptic location and function of the prion protein. J Neurosci 1999; 19:8866 - 8875
   PubMed Web of Science ®Google Scholar
• Haeberlé AM, Ribaut-Barassin C, Bombarde G, Mariani J, Hunsmann G, Grassi J, et al. Synaptic prion protein immuno-reactivity in the rodent cerebellum. Microsc Res Tech 2000; 50:66 - 75
   PubMed Web of Science ®Google Scholar
• Kanaani J, Prusiner SB, Diacovo J, Baekkeskov S, Legname G. Recombinant prion protein induces rapid polarization and development of synapses in embryonic rat hippocampal neurons in vitro. J Neurochem 2005; 95:1373 - 1386
   PubMed Web of Science ®Google Scholar
• Jeffrey M, Halliday WG, Bell J, Johnston AR, MacLeod NK, Ingham C, et al. Synapse loss associated with abnormal PrP precedes neuronal degeneration in the scrapie-infected murine hippocampus. Neuropathol Appl Neurobiol 2000; 26:41 - 54
   PubMed Web of Science ®Google Scholar
• Collinge J, Whittington MA, Sidle KC, Smith CJ, Palmer MS, Clarke AR, et al. Prion protein is necessary for normal synaptic function. Nature 1994; 370:295 - 297
   PubMed Web of Science ®Google Scholar
• Colling SB, Collinge J, Jefferys JG. Hippocampal slices from prion protein null mice: disrupted Ca(2+)-activated K+ currents. Neurosci Lett 1996; 209:49 - 52
   PubMed Web of Science ®Google Scholar
• Coitinho AS, Roesler R, Martins VR, Brentani RR, Izquierdo I. Cellular prion protein ablation impairs behavior as a function of age. Neuroreport 2003; 14:1375 - 1379
   PubMed Web of Science ®Google Scholar
• Criado JR, Sánchez-Alavez M, Conti B, Giacchino JL, Wills DN, Henriksen SJ, et al. Mice devoid of prion protein have cognitive deficits that are rescued by reconstitution of PrP in neurons. Neurobiol Dis 2005; 19:255 - 265
   PubMed Web of Science ®Google Scholar
• Carleton A, Tremblay P, Vincent JD, Lledo PM. Dose-dependent, prion protein (PrP)-mediated facilitation of excitatory synaptic transmission in the mouse hippocampus. Pflugers Arch 2001; 442:223 - 229
   PubMed Web of Science ®Google Scholar
• Coitinho AS, Lopes MH, Hajj GN, Rossato JI, Freitas AR, Castro CC, et al. Short-term memory formation and long-term memory consolidation are enhanced by cellular prion association to stress-inducible protein 1. Neurobiol Dis 2007; 26:282 - 290
   PubMed Web of Science ®Google Scholar
• Coitinho AS, Freitas AR, Lopes MH, Hajj GN, Roesler R, Walz R, et al. The interaction between prion protein and laminin modulates memory consolidation. Eur J Neurosci 2006; 24:3255 - 3264
   PubMed Web of Science ®Google Scholar
• Parkin ET, Watt NT, Hussain I, Eckman EA, Eckman CB, Manson JC, et al. Cellular prion protein regulates beta-secretase cleavage of the Alzheimer's amyloid precursor protein. Proc Natl Acad Sci USA 2007; 104:11062 - 11067
   PubMed Web of Science ®Google Scholar
• Mallucci G, Dickinson A, Linehan J, Klöhn PC, Brandner S, Collinge J. Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis. Science 2003; 302:871 - 874
   PubMed Web of Science ®Google Scholar
• Mallucci GR, White MD, Farmer M, Dickinson A, Khatun H, Powell AD, et al. Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected mice. Neuron 2007; 53:325 - 335
   PubMed Web of Science ®Google Scholar
• Enari M, Flechsig E, Weissmann C. Scrapie prion protein accumulation by scrapie-infected neuroblastoma cells abrogated by exposure to a prion protein antibody. Proc Natl Acad Sci USA 2001; 98:9295 - 9299
   PubMed Web of Science ®Google Scholar
• White AR, Enever P, Tayebi M, Mushens R, Linehan J, Brandner S, Hawke S, et al. Monoclonal antibodies inhibit prion replication and delay the development of Prion disease. Nature 2003; 422:80 - 83
   PubMed Web of Science ®Google Scholar
• Zhou X, Bi H, Wong J, Shimoji M, Yuan J, Xiao X, et al. Alkylating antitumor drug mechlorethamine conceals a structured PrP domain and inhibits in vitro prion amplification. J Toxicolo Environ Health 2011; In press
   Web of Science ®Google Scholar
• Büeler H, Aguzzi A, Sailer A, Greiner RA, Autenried P, Aguet M, et al. Mice devoid of PrP are resistant to scrapie. Cell 1993; 73:1339 - 1347
   PubMed Web of Science ®Google Scholar
• Berr C, Helbecque N, Sazdovitch V, Mohr M, Amant C, Amouyel P, et al. Polymorphism of the codon 129 of the prion protein (PrP) gene and neuropathology of cerebral ageing. Acta Neuropathol 2003; 106:71 - 74
   Google Scholar
• Laurén J, Gimbel DA, Nygaard HB, Gilbert JW, Strittmatter SM. Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers. Nature 2009; 457:1128 - 1132
   PubMed Web of Science ®Google Scholar
• Gimbel DA, Nygaard HB, Coffey EE, Gunther EC, Laurén J, Gimbel ZA, et al. Memory impairment in transgenic Alzheimer mice requires cellular prion protein. J Neurosci 2010; 30:6367 - 6374
   PubMed Web of Science ®Google Scholar
• Balducci C, Beeg M, Stravalaci M, Bastone A, Sclip A, Biasini E, et al. Synthetic amyloid-beta oligomers impair long-term memory independently of cellular prion protein. Proc Natl Acad Sci USA 2010; 107:2295 - 2300
   PubMed Web of Science ®Google Scholar
• Calella AM, Farinelli M, Nuvolone M, Mirante O, Moos R, Falsig J, et al. Prion protein and Abeta-related synaptic toxicity impairment. EMBO Mol Med 2010; 2:306 - 314
   PubMed Web of Science ®Google Scholar
• Kessels HW, Nguyen LN, Nabavi S, Malinow R. The prion protein as a receptor for amyloid-beta. Nature 2010; 466:3 - 4
   PubMed Web of Science ®Google Scholar
• Chung E, Ji Y, Sun Y, Kascsak RJ, Kascsak RB, Mehta PD, et al. Anti-PrPC monoclonal antibody infusion as a novel treatment for cognitive deficits in an Alzheimer's disease model mouse. BMC Neurosci 2010; 11:130
   PubMed Web of Science ®Google Scholar
• Barry AE, Klyubin I, Mc Donald JM, Mably AJ, Farrell MA, Scott M, et al. Alzheimer's Disease Brain-Derived Amyloid-{beta}-Mediated Inhibition of LTP In Vivo Is Prevented by Immunotargeting Cellular Prion Protein. J Neurosci 2011; 31:7259 - 7263
   PubMed Web of Science ®Google Scholar
• Resenberger UK, Harmeier A, Woerner AC, Goodman JL, Müller V, Krishnan R, et al. The cellular prion protein mediates neurotoxic signalling of β-sheet-rich conformers independent of prion replication. EMBO J 2011; 30:2057 - 2070
   PubMed Web of Science ®Google Scholar
• Zou WQ, Xiao X, Yuan J, Puoti G, Fujioka H, Wang X, et al. Amyloid-{beta}42 Interacts Mainly with Insoluble Prion Protein in the Alzheimer Brain. J Biol Chem 2011; 286:15095 - 15105
   PubMed Web of Science ®Google Scholar
• Westaway D, Alier K, Vergote D, MacTavish D, Mercer R, Fu W, et al. Prion proteins and the Alzheimer disease Aβ amyloid cascade. Prion 2011; 5:1
   PubMed Web of Science ®Google Scholar
• Fleisch VC, Kaiser DM, Leighton P, Wang H, Allision WT. A novel genetic interaction between APP and PrP: notorious proteins conspire during cell adhesion in a pathway leading to CNS cell death. Prion 2011; 5:112
   Web of Science ®Google Scholar
• Schmitz M. Influence of PrPC on the expression of Alzheimer disease-relevant proteins. Prion 2011; 5:120
   Web of Science ®Google Scholar
• Wasilewska-Sampaio AP, Hirata P, Prado M, Brito-Moreira J, Ferreira S, Hajj G, et al. PrPC-STI1 interaction protects against synapse damage caused by soluble Aβ oligomers. Prion 2011; 5:121
   Web of Science ®Google Scholar
• Zou WQ. Chameleon-like prion protein and human cognition. Current Topics in Biochemical Research 2010; 12:1 - 8
   Google Scholar
• Zhang H, Stockel J, Mehlhorn I, Groth D, Baldwin MA, Prusiner SB, et al. Physical studies of conformational plasticity in a recombinant prion protein. Biochemistry 1997; 36:3543 - 3553
   Google Scholar
• Nicholson EM, Mo H, Prusiner SB, Cohen FE, Marqusee S. Differences between the prion protein and its homolog Doppel: a partially structured state with implications for scrapie formation. J Mol Biol 2002; 316:807 - 815
   PubMed Web of Science ®Google Scholar
• Kuwata K, Li H, Yamada H, Legname G, Prusiner SB, Akasaka K, et al. Locally disordered conformer of the hamster prion protein: a crucial intermediate to PrPSc?. Biochemistry 2002; 41:12277 - 12283
   PubMed Web of Science ®Google Scholar
• Martins SM, Chapeaurouge A, Ferreira ST. Folding intermediates of the prion protein stabilized by hydrostatic pressure and low temperature. J Biol Chem 2003; 278:50449 - 50455
   PubMed Web of Science ®Google Scholar
• Baskakov IV, Legname G, Prusiner SB, Cohen FE. Folding of prion protein to its native alpha-helical conformation is under kinetic control. J Biol Chem 2001; 276:19687 - 19690
   PubMed Web of Science ®Google Scholar
• Morillas M, Vanik DL, Surewicz WK. On the mechanism of alpha-helix to beta-sheet transition in the recombinant prion protein. Biochemistry 2001; 40:6982 - 6987
   PubMed Web of Science ®Google Scholar
• Lu BY, Chang JY. Isolation and characterization of a polymerized prion protein. Biochem J 2002; 364:81 - 87
   PubMed Web of Science ®Google Scholar
• Sokolowski F, Modler AJ, Masuch R, Zirwer D, Baier M, Lutsch G, et al. Formation of critical oligomers is a key event during conformational transition of recombinant syrian hamster prion protein. J Biol Chem 2003; 278:40481 - 40492
   PubMed Web of Science ®Google Scholar
• Baskakov IV, Legname G, Gryczynski Z, Prusiner SB. The peculiar nature of unfolding of the human prion protein. Protein Sci 2004; 13:586 - 595
   PubMed Web of Science ®Google Scholar
• Baskakov IV, Legname G, Baldwin MA, Prusiner SB, Cohen FE. Pathway complexity of prion protein assembly into amyloid. J Biol Chem 2002; 277:21140 - 21148
   PubMed Web of Science ®Google Scholar
• Zou WQ, Zheng J, Gray DM, Gambetti P, Chen SG. Antibody to DNA detects scrapie but not normal prion protein. Proc Natl Acad Sci USA 2004; 101:1380 - 1385
   PubMed Web of Science ®Google Scholar
• Strom A, Wang GS, Reimer R, Finegood DT, Scott FW. Pronounced cytosolic aggregation of cellular prion protein in pancreatic beta-cells in response to hyperglycemia. Lab Invest 2007; 87:139 - 149
   PubMed Web of Science ®Google Scholar
• Kuczius T, Karch H, Groschup MH. Differential solubility of prions is associated in manifold phenotypes. Mol Cell Neurosci 2009; 42:226 - 233
   PubMed Web of Science ®Google Scholar
• Paramithiotis E, Pinard M, Lawton T, LaBoissiere S, Leathers VL, Zou WQ, et al. A prion protein epitope selective for the pathologically misfolded conformation. Nat Med 2003; 9:893 - 899
   PubMed Web of Science ®Google Scholar
• Biasini E, Seegulam ME, Patti BN, Solforosi L, Medrano AZ, Christensen HM, et al. Non-infectious aggregates of the prion protein react with several PrPSc-directed antibodies. J Neurochem 2008; 105:2190 - 2204
   Google Scholar
• Biasini E, Tapella L, Mantovani S, Stravalaci M, Gobbi M, Harris DA, et al. Immunopurification of pathological prion protein aggregates. PLoS One 2009; 4:7816
   PubMed Web of Science ®Google Scholar
• Pan T, Li R, Wong BS, Liu T, Gambetti P, Sy MS. Heterogeneity of normal prion protein in two-dimensional immunoblot: presence of various glycosylated and truncated forms. J Neurochem 2002; 81:1092 - 1101
   PubMed Web of Science ®Google Scholar
• Beringue V, Mallinson G, Kaisar M, Tayebi M, Sattar Z, Jackson G, et al. Regional heterogeneity of cellular prion protein isoforms in the mouse brain. Brain 2003; 126:2065 - 2073
   PubMed Web of Science ®Google Scholar
• Safar JG, DeArmond SJ, Kociuba K, Deering C, Didorenko S, Bouzamondo-Bernstein E, et al. Prion clearance in bigenic mice. J Gen Virol 2005; 86:2913 - 2923
   PubMed Web of Science ®Google Scholar
• Barria MA, Mukherjee A, Gonzalez-Romero D, Morales R, Soto C. De novo generation of infectious prions in vitro produces a new disease phenotype. PLoS Pathog 2009; 5:1000421
   PubMed Web of Science ®Google Scholar
• Westaway D, DeArmond SJ, Cayetano-Canlas J, Groth D, Foster D, Yang SL, et al. Degeneration of skeletal muscle, peripheral nerves and the central nervous system in transgenic mice overexpressing wild-type prion proteins. Cell 1994; 76:117 - 129
   PubMed Web of Science ®Google Scholar
• Chiesa R, Piccardo P, Biasini E, Ghetti B, Harris DA. Aggregated, wild-type prion protein causes neurological dysfunction and synaptic abnormalities. J Neurosci 2008; 28:13258 - 13267
   PubMed Web of Science ®Google Scholar
• Fernandez-Funez P, Zhang Y, Casas-Tinto S, Xiao X, Zou WQ, Rincon-Limas DE. Sequence-dependent prion protein misfolding and neurotoxicity. J Biol Chem 2010; 285:36897 - 36908
   PubMed Web of Science ®Google Scholar
• Yuan J, Dong Z, Guo JP, McGeehan J, Xiao X, Wang J, et al. Accessibility of a critical prion protein region involved in strain recognition and its implications for the early detection of prions. Cell Mol Life Sci 2008; 65:631 - 643
   PubMed Web of Science ®Google Scholar
• Zou WQ, Langeveld J, Xiao X, Chen S, McGeer PL, Yuan J, et al. PrP conformational transitions alter species preference of a PrP-specific antibody. J Biol Chem 2010; 285:13874 - 13884
   PubMed Web of Science ®Google Scholar
• Gambetti P, Dong Z, Yuan J, Xiao X, Zheng M, Alshekhlee A, et al. A novel human disease with abnormal prion protein sensitive to protease. Ann Neurol 2008; 63:697 - 708
   PubMed Web of Science ®Google Scholar
• Zou WQ, Puoti G, Xiao X, Yuan J, Qing L, Cali I, et al. Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein. Ann Neurol 2010; 68:162 - 172
   PubMed Web of Science ®Google Scholar
• Gambetti P, Puoti G, Zou WQ. Variably Protease-Sensitive Prionopathy: A novel Disease of the Prion Protein. J Mol Neurosci 2011; May 17 [Epub ahead of print]
   PubMed Web of Science ®Google Scholar
• Gambetti P, Zou WQ, Torres JM, Soto C, Notari S, Espinosa JC, et al. Variably protease-sensitive prionopathy: Transmissibility and PMCA studies. Prion 2011; 5:14
   Web of Science ®Google Scholar
• Zou WQ, Xiao X, Yuan J, Puoti G, Fujioka H, Wang X, et al. Amyloid-{beta}42 Interacts Mainly with Insoluble Prion Protein in the Alzheimer Brain. J Biol Chem 2011; 286:15095 - 15105
   PubMed Web of Science ®Google Scholar
• Chen S, Yadav SP, Surewicz WK. Interaction between human prion protein and amyloid-beta (Abeta) oligomers: role OF N-terminal residues. J Biol Chem 2010; 285:26377 - 26383
   PubMed Web of Science ®Google Scholar
• Esiri MM, Carter J, Ironside JW. Prion protein immunoreactivity in brain samples from an unselected autopsy population: findings in 200 consecutive cases. Neuropathol Appl Neurobiol 2000; 26:273 - 284
   PubMed Web of Science ®Google Scholar
• Ferrer I, Blanco R, Carmona M, Puig B, Ribera R, Rey MJ, et al. Prion protein expression in senile plaques in Alzheimer's disease. Acta Neuropathol 2001; 101:49 - 56
   PubMed Web of Science ®Google Scholar
• Kovacs GG, Zerbi P, Voigtländer T, Strohschneider M, Trabattoni G, Hainfellner JA, et al. The prion protein in human neurodegenerative disorders. Neurosci Lett 2002; 329:269 - 272
   PubMed Web of Science ®Google Scholar
• Morales R, Estrada LD, Diaz-Espinoza R, Morales-Scheihing D, Jara MC, Castilla J, et al. Molecular cross talk between misfolded proteins in animal models of Alzheimer's and Prion diseases. J Neurosci 2010; 30:4528 - 4535
   PubMed Web of Science ®Google Scholar
• Schwarze-Eicker K, Keyvani K, Görtz N, Westaway D, Sachser N, Paulus W. Prion protein (PrPc) promotes beta-amyloid plaque formation. Neurobiol Aging 2005; 26:1177 - 1182
   PubMed Web of Science ®Google Scholar
• Giasson BI, Forman MS, Higuchi M, Golbe LI, Graves CL, Kotzbauer PT, et al. Initiation and synergistic fibrillization of tau and alpha-synuclein. Science 2003; 300:636 - 640
   PubMed Web of Science ®Google Scholar
• Si K, Giustetto M, Etkin A, Hsu R, Janisiewicz AM, Miniaci MC, et al. A neuronal isoform of CPEB regulates local protein synthesis and stabilizes synapse-specific long-term facilitation in aplysia. Cell 2003; 115:893 - 904
   PubMed Web of Science ®Google Scholar
• Si K, Lindquist S, Kandel ER. A neuronal isoform of the aplysia CPEB has prion-like properties. Cell 2003; 115:879 - 891
   PubMed Web of Science ®Google Scholar
• Si K, Choi YB, White-Grindley E, Majumdar A, Kandel ER. Aplysia CPEB can form prion-like multimers in sensory neurons that contribute to long-term facilitation. Cell 2010; 140:421 - 435
   PubMed Web of Science ®Google Scholar
• Papassotiropoulos A, Wollmer MA, Aguzzi A, Hock C, Nitsch RM, de Quervain DJ. The prion gene is associated with human long-term memory. Hum Mol Genet 2005; 14:2241 - 2246
   PubMed Web of Science ®Google Scholar
• Shorter J, Lindquist S. Prions as adaptive conduits of memory and inheritance. Nat Rev Genet 2005; 6:435 - 450
   PubMed Web of Science ®Google Scholar
• Tompa P, Friedrich P. Prion proteins as memory molecules: an hypothesis. Neuroscience 1998; 86:1037 - 1043
   PubMed Web of Science ®Google Scholar
• Zou WQ. Transmissible spongiform encephalopathy and beyond (E-letter). Science http://www.sciencemag.org/content/308/5727/1420.long/reply#sci_el_10316, 20, Sep 2007
   Google Scholar