Note to users. If you're seeing this message, it means that your browser cannot find this page's style/presentation instructions -- or possibly that you are using a browser that does not support current Web standards. Find out more about why this message is appearing, and what you can do to make your experience of our site the best it can be.


Science 14 June 1991:
Vol. 252. no. 5012, pp. 1515 - 1522
DOI: 10.1126/science.1675487

Articles

Science, Vol 252, Issue 5012, 1515-1522
Copyright © 1991 by American Association for the Advancement of Science


articles

Molecular biology of prion diseases

SB Prusiner

Department of Neurology, University of California, San Francisco 94143.

Prions cause transmissible and genetic neurodegenerative diseases, including scrapie and bovine spongiform encephalopathy of animals and Creutzfeldt-Jakob and Gerstmann-Straussler-Scheinker diseases of humans. Infectious prion particles are composed largely, if not entirely, of an abnormal isoform of the prion protein, which is encoded by a chromosomal gene. A posttranslational process, as yet unidentified, converts the cellular prion protein into an abnormal isoform. Scrapie incubation times, neuropathology, and prion synthesis in transgenic mice are controlled by the prion protein gene. Point mutations in the prion protein genes of animals and humans are genetically linked to development of neuro-degeneration. Transgenic mice expressing mutant prion proteins spontaneously develop neurologic dysfunction and spongiform neuropathology. Understanding prion diseases may advance investigations of other neurodegenerative disorders and of the processes by which neurons differentiate, function for decades, and then grow senescent.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Prion-induced Activation of Cholesterogenic Gene Expression by Srebp2 in Neuronal Cells.
C. Bach, S. Gilch, R. Rost, A. D. Greenwood, M. Horsch, G. N. M. Hajj, S. Brodesser, A. Facius, S. Schadler, K. Sandhoff, et al. (2009)
J. Biol. Chem. 284, 31260-31269
   Abstract »    Full Text »    PDF »
Analysis of Protein Levels of 24 Cytokines in Scrapie Agent-Infected Brain and Glial Cell Cultures from Mice Differing in Prion Protein Expression Levels.
D. Tribouillard-Tanvier, J. F. Striebel, K. E. Peterson, and B. Chesebro (2009)
J. Virol. 83, 11244-11253
   Abstract »    Full Text »    PDF »
Alternative Translation Initiation Generates Cytoplasmic Sheep Prion Protein.
C. Lund, C. M. Olsen, S. Skogtvedt, H. Tveit, K. Prydz, and M. A. Tranulis (2009)
J. Biol. Chem. 284, 19668-19678
   Abstract »    Full Text »    PDF »
Doxorubicin and Congo Red Effectiveness on Prion Infectivity in Golden Syrian Hamster.
M. CORATO, P. OGLIARI, F. CECILIANI, E. COVA, V. BELLOTTI, C. CEREDA, G. MERLINI, and M. CERONI (2009)
Anticancer Res 29, 2507-2512
   Abstract »    Full Text »    PDF »
Design of anti- and pro-aggregation variants to assess the effects of methionine oxidation in human prion protein.
C. Wolschner, A. Giese, H. A. Kretzschmar, R. Huber, L. Moroder, and N. Budisa (2009)
PNAS 106, 7756-7761
   Abstract »    Full Text »    PDF »
Highly sensitive, quantitative cell-based assay for prions adsorbed to solid surfaces.
J. A. Edgeworth, G. S. Jackson, A. R. Clarke, C. Weissmann, and J. Collinge (2009)
PNAS 106, 3479-3483
   Abstract »    Full Text »    PDF »
Alteration of the biological and biochemical characteristics of bovine spongiform encephalopathy prions during interspecies transmission in transgenic mice models.
T. Yokoyama, K. Masujin, Y. Iwamaru, M. Imamura, and S. Mohri (2009)
J. Gen. Virol. 90, 261-268
   Abstract »    Full Text »    PDF »
Prion Strain Targeting Independent of Strain-Specific Neuronal Tropism.
J. I. Ayers, A. E. Kincaid, and J. C. Bartz (2009)
J. Virol. 83, 81-87
   Abstract »    Full Text »    PDF »
Identifying Key Components of the PrPC-PrPSc Replicative Interface.
G. C. Abalos, J. T. Cruite, A. Bellon, S. Hemmers, J. Akagi, J. A. Mastrianni, R. A. Williamson, and L. Solforosi (2008)
J. Biol. Chem. 283, 34021-34028
   Abstract »    Full Text »    PDF »
Histidine at codon 154 of the prion protein gene is a risk factor for Nor98 scrapie in goats.
S. Colussi, G. Vaccari, C. Maurella, C. Bona, R. Lorenzetti, P. Troiano, F. Casalinuovo, A. Di Sarno, M. G. Maniaci, F. Zuccon, et al. (2008)
J. Gen. Virol. 89, 3173-3176
   Abstract »    Full Text »    PDF »
In vitro amplification of PrPSc derived from the brain and blood of sheep infected with scrapie.
L. Thorne and L. A. Terry (2008)
J. Gen. Virol. 89, 3177-3184
   Abstract »    Full Text »    PDF »
The Mad2 partial unfolding model: regulating mitosis through Mad2 conformational switching.
J. J. Skinner, S. Wood, J. Shorter, S. W. Englander, and B. E. Black (2008)
J. Cell Biol. 183, 761-768
   Abstract »    Full Text »    PDF »
Structural changes of membrane-anchored native PrPC.
K. Elfrink, J. Ollesch, J. Stohr, D. Willbold, D. Riesner, and K. Gerwert (2008)
PNAS 105, 10815-10819
   Abstract »    Full Text »    PDF »
Prion strain discrimination in cell culture: The cell panel assay.
S. P. Mahal, C. A. Baker, C. A. Demczyk, E. W. Smith, C. Julius, and C. Weissmann (2007)
PNAS 104, 20908-20913
   Abstract »    Full Text »    PDF »
Prion propagation in a nerve conduit model containing segments devoid of axons.
C. Kratzel, D. Kruger, and M. Beekes (2007)
J. Gen. Virol. 88, 3479-3485
   Abstract »    Full Text »    PDF »
Orally Administered Amyloidophilic Compound Is Effective in Prolonging the Incubation Periods of Animals Cerebrally Infected with Prion Diseases in a Prion Strain-Dependent Manner.
Y. Kawasaki, K. Kawagoe, C.-j. Chen, K. Teruya, Y. Sakasegawa, and K. Doh-ura (2007)
J. Virol. 81, 12889-12898
   Abstract »    Full Text »    PDF »
From the Cover: Cell division modulates prion accumulation in cultured cells.
S. Ghaemmaghami, P.-W. Phuan, B. Perkins, J. Ullman, B. C. H. May, F. E. Cohen, and S. B. Prusiner (2007)
PNAS 104, 17971-17976
   Abstract »    Full Text »    PDF »
Cyclodextrins Inhibit Replication of Scrapie Prion Protein in Cell Culture.
M. Prior, S. Lehmann, M.-S. Sy, B. Molloy, and H. E. M. McMahon (2007)
J. Virol. 81, 11195-11207
   Abstract »    Full Text »    PDF »
Urinary excretion and blood level of prions in scrapie-infected hamsters.
Y. Murayama, M. Yoshioka, H. Okada, M. Takata, T. Yokoyama, and S. Mohri (2007)
J. Gen. Virol. 88, 2890-2898
   Abstract »    Full Text »    PDF »
Reduced susceptibility to bovine spongiform encephalopathy prions in transgenic mice expressing a bovine PrP with five octapeptide repeats.
A. Brun, A. Gutierrez-Adan, J. Castilla, B. Pintado, F. Diaz-San Segundo, M. J. Cano, E. Alamillo, J. C. Espinosa, and J. M. Torres (2007)
J. Gen. Virol. 88, 1842-1849
   Abstract »    Full Text »    PDF »
Prions in the peripheral nerves of bovine spongiform encephalopathy-affected cattle.
K. Masujin, D. Matthews, G. A. H. Wells, S. Mohri, and T. Yokoyama (2007)
J. Gen. Virol. 88, 1850-1858
   Abstract »    Full Text »    PDF »
Human prion proteins with pathogenic mutations share common conformational changes resulting in enhanced binding to glycosaminoglycans.
S. Yin, N. Pham, S. Yu, C. Li, P. Wong, B. Chang, S.-C. Kang, E. Biasini, P. Tien, D. A. Harris, et al. (2007)
PNAS 104, 7546-7551
   Abstract »    Full Text »    PDF »
Neurotoxic and Gliotrophic Activity of a Synthetic Peptide Homologous to Gerstmann-Straussler-Scheinker Disease Amyloid Protein.
L. Fioriti, N. Angeretti, L. Colombo, A. De Luigi, A. Colombo, C. Manzoni, M. Morbin, F. Tagliavini, M. Salmona, R. Chiesa, et al. (2007)
J. Neurosci. 27, 1576-1583
   Abstract »    Full Text »    PDF »
Sheep-Passaged Bovine Spongiform Encephalopathy Agent Exhibits Altered Pathobiological Properties in Bovine-PrP Transgenic Mice.
J. C. Espinosa, O. Andreoletti, J. Castilla, M. E. Herva, M. Morales, E. Alamillo, F. D. San-Segundo, C. Lacroux, S. Lugan, F. J. Salguero, et al. (2007)
J. Virol. 81, 835-843
   Abstract »    Full Text »    PDF »
Prion infectivity in variant Creutzfeldt-Jakob disease rectum.
J D F Wadsworth, S Joiner, K Fox, J M Linehan, M Desbruslais, S Brandner, E A Asante, and J Collinge (2007)
Gut 56, 90-94
   Abstract »    Full Text »    PDF »
A case-control study of scrapie Nor98 in Norwegian sheep flocks.
P. Hopp, M. K. Omer, and B. T. Heier (2006)
J. Gen. Virol. 87, 3729-3736
   Abstract »    Full Text »    PDF »
A novel, resistance-linked ovine PrP variant and its equivalent mouse variant modulate the in vitro cell-free conversion of rPrP to PrPres.
L. Kirby, W. Goldmann, F. Houston, A. C. Gill, and J. C. Manson (2006)
J. Gen. Virol. 87, 3747-3751
   Abstract »    Full Text »    PDF »
Insoluble Aggregates and Protease-resistant Conformers of Prion Protein in Uninfected Human Brains.
J. Yuan, X. Xiao, J. McGeehan, Z. Dong, I. Cali, H. Fujioka, Q. Kong, G. Kneale, P. Gambetti, and W.-Q. Zou (2006)
J. Biol. Chem. 281, 34848-34858
   Abstract »    Full Text »    PDF »
Prion Proteins with Insertion Mutations Have Altered N-terminal Conformation and Increased Ligand Binding Activity and Are More Susceptible to Oxidative Attack.
S. Yin, S. Yu, C. Li, P. Wong, B. Chang, F. Xiao, S.-C. Kang, H. Yan, G. Xiao, J. Grassi, et al. (2006)
J. Biol. Chem. 281, 10698-10705
   Abstract »    Full Text »    PDF »
Prion infection of mouse neurospheres.
R. K. Giri, R. Young, R. Pitstick, S. J. DeArmond, S. B. Prusiner, and G. A. Carlson (2006)
PNAS 103, 3875-3880
   Abstract »    Full Text »    PDF »
Transmission of Amyloidosis in Offspring of Mice with AApoAII Amyloidosis.
T. Korenaga, J. Yan, J. Sawashita, T. Matsushita, H. Naiki, M. Hosokawa, M. Mori, K. Higuchi, and X. Fu (2006)
Am. J. Pathol. 168, 898-906
   Abstract »    Full Text »    PDF »
The prion protein gene in humans revisited: Lessons from a worldwide resequencing study.
M. Soldevila, A. M. Andres, A. Ramirez-Soriano, T. Marques-Bonet, F. Calafell, A. Navarro, and J. Bertranpetit (2006)
Genome Res. 16, 231-239
   Abstract »    Full Text »    PDF »
Gerstmann-Straussler-Scheinker Disease Amyloid Protein Polymerizes According to the "Dock-and-Lock" Model.
M. Gobbi, L. Colombo, M. Morbin, G. Mazzoleni, E. Accardo, M. Vanoni, E. Del Favero, L. Cantu, D. A. Kirschner, C. Manzoni, et al. (2006)
J. Biol. Chem. 281, 843-849
   Abstract »    Full Text »    PDF »
Absence of Spiroplasma or Other Bacterial 16S rRNA Genes in Brain Tissue of Hamsters with Scrapie.
I. Alexeeva, E. J. Elliott, S. Rollins, G. E. Gasparich, J. Lazar, and R. G. Rohwer (2006)
J. Clin. Microbiol. 44, 91-97
   Abstract »    Full Text »    PDF »
Potential Role of Soil in the Transmission of Prion Disease.
P. T. Schramm, C. J. Johnson, N. E. Mathews, D. McKenzie, J. M. Aiken, and J. A. Pedersen (2006)
Reviews in Mineralogy and Geochemistry 64, 135-152
   Full Text »    PDF »
Overexpression of PrPC by Adenovirus-Mediated Gene Targeting Reduces Ischemic Injury in a Stroke Rat Model.
W.-C. Shyu, S.-Z. Lin, M.-F. Chiang, D.-C. Ding, K.-W. Li, S.-F. Chen, H.-I Yang, and H. Li (2005)
J. Neurosci. 25, 8967-8977
   Abstract »    Full Text »    PDF »
PrP glycoforms are associated in a strain-specific ratio in native PrPSc.
A. Khalili-Shirazi, L. Summers, J. Linehan, G. Mallinson, D. Anstee, S. Hawke, G. S. Jackson, and J. Collinge (2005)
J. Gen. Virol. 86, 2635-2644
   Abstract »    Full Text »    PDF »
Role of Plasminogen in Propagation of Scrapie.
M. Salmona, R. Capobianco, L. Colombo, A. De Luigi, G. Rossi, M. Mangieri, G. Giaccone, E. Quaglio, R. Chiesa, M. B. Donati, et al. (2005)
J. Virol. 79, 11225-11230
   Abstract »    Full Text »    PDF »
Search for a Prion-Specific Nucleic Acid.
J. G. Safar, K. Kellings, A. Serban, D. Groth, J. E. Cleaver, S. B. Prusiner, and D. Riesner (2005)
J. Virol. 79, 10796-10806
   Abstract »    Full Text »    PDF »
Elimination of transmissible spongiform encephalopathy infectivity and decontamination of surgical instruments by using radio-frequency gas-plasma treatment.
H. C. Baxter, G. A. Campbell, A. G. Whittaker, A. C. Jones, A. Aitken, A. H. Simpson, M. Casey, L. Bountiff, L. Gibbard, and R. L. Baxter (2005)
J. Gen. Virol. 86, 2393-2399
   Abstract »    Full Text »    PDF »
Molecular neurology of prion disease.
J Collinge (2005)
J. Neurol. Neurosurg. Psychiatry 76, 906-919
   Abstract »    Full Text »    PDF »
Beyond the Signal Sequence: Protein Routing in Health and Disease.
C. Castro-Fernandez, G. Maya-Nunez, and P. M. Conn (2005)
Endocr. Rev. 26, 479-503
   Abstract »    Full Text »    PDF »
Screening of 145 Anti-PrP Monoclonal Antibodies for Their Capacity to Inhibit PrPSc Replication in Infected Cells.
C. Feraudet, N. Morel, S. Simon, H. Volland, Y. Frobert, C. Creminon, D. Vilette, S. Lehmann, and J. Grassi (2005)
J. Biol. Chem. 280, 11247-11258
   Abstract »    Full Text »    PDF »
Protein Conformation Significantly Influences Immune Responses to Prion Protein.
A. Khalili-Shirazi, S. Quaratino, M. Londei, L. Summers, M. Tayebi, A. R. Clarke, S. H. Hawke, G. S. Jackson, and J. Collinge (2005)
J. Immunol. 174, 3256-3263
   Abstract »    Full Text »    PDF »
Abnormal prion protein in genetically resistant sheep from a scrapie-infected flock.
J.-Y. Madec, S. Simon, S. Lezmi, A. Bencsik, J. Grassi, and T. Baron (2004)
J. Gen. Virol. 85, 3483-3486
   Abstract »    Full Text »    PDF »
Prion protein gene (PRNP) variants and evidence for strong purifying selection in functionally important regions of bovine exon 3.
C. M. Seabury, R. L. Honeycutt, A. P. Rooney, N. D. Halbert, and J. N. Derr (2004)
PNAS 101, 15142-15147
   Abstract »    Full Text »    PDF »
Role of Microglia in Central Nervous System Infections.
R. B. Rock, G. Gekker, S. Hu, W. S. Sheng, M. Cheeran, J. R. Lokensgard, and P. K. Peterson (2004)
Clin. Microbiol. Rev. 17, 942-964
   Abstract »    Full Text »    PDF »
Characterization of two distinct prion strains derived from bovine spongiform encephalopathy transmissions to inbred mice.
S. E. Lloyd, J. M. Linehan, M. Desbruslais, S. Joiner, J. Buckell, S. Brandner, J. D. F. Wadsworth, and J. Collinge (2004)
J. Gen. Virol. 85, 2471-2478
   Abstract »    Full Text »    PDF »
Methionine 129 Variant of Human Prion Protein Oligomerizes More Rapidly than the Valine 129 Variant: IMPLICATIONS FOR DISEASE SUSCEPTIBILITY TO CREUTZFELDT-JAKOB DISEASE.
A. Tahiri-Alaoui, A. C. Gill, P. Disterer, and W. James (2004)
J. Biol. Chem. 279, 31390-31397
   Abstract »    Full Text »    PDF »
Standards for the assay of Creutzfeldt-Jakob disease specimens.
P. Minor, J. Newham, N. Jones, C. Bergeron, L. Gregori, D. Asher, F. van Engelenburg, T. Stroebel, M. Vey, G. Barnard, et al. (2004)
J. Gen. Virol. 85, 1777-1784
   Abstract »    Full Text »    PDF »
Amyloid imaging probes are useful for detection of prion plaques and treatment of transmissible spongiform encephalopathies.
K. Ishikawa, K. Doh-ura, Y. Kudo, N. Nishida, I. Murakami-Kubo, Y. Ando, T. Sawada, and T. Iwaki (2004)
J. Gen. Virol. 85, 1785-1790
   Abstract »    Full Text »    PDF »
Subclinical Bovine Spongiform Encephalopathy Infection in Transgenic Mice Expressing Porcine Prion Protein.
J. Castilla, A. Gutierrez-Adan, A. Brun, D. Doyle, B. Pintado, M. A. Ramirez, F. J. Salguero, B. Parra, F. D. S. Segundo, J. M. Sanchez-Vizcaino, et al. (2004)
J. Neurosci. 24, 5063-5069
   Abstract »    Full Text »    PDF »
Tissue Distribution, Biochemical Properties, and Transmission of Mouse Type A AApoAII Amyloid Fibrils.
T. Korenaga, X. Fu, Y. Xing, T. Matsusita, K. Kuramoto, S. Syumiya, K. Hasegawa, H. Naiki, M. Ueno, T. Ishihara, et al. (2004)
Am. J. Pathol. 164, 1597-1606
   Abstract »    Full Text »    PDF »
Different Behavior toward Bovine Spongiform Encephalopathy Infection of Bovine Prion Protein Transgenic Mice with One Extra Repeat Octapeptide Insert Mutation.
J. Castilla, A. Gutierrez-Adan, A. Brun, B. Pintado, B. Parra, M. A. Ramirez, F. J. Salguero, F. Diaz San Segundo, A. Rabano, M. J. Cano, et al. (2004)
J. Neurosci. 24, 2156-2164
   Abstract »    Full Text »    PDF »
From conversion to aggregation: Protofibril formation of the prion protein.
M. L. DeMarco and V. Daggett (2004)
PNAS 101, 2293-2298
   Abstract »    Full Text »    PDF »
Quinoline Derivatives Are Therapeutic Candidates for Transmissible Spongiform Encephalopathies.
I. Murakami-Kubo, K. Doh-ura, K. Ishikawa, S. Kawatake, K. Sasaki, J.-i. Kira, S. Ohta, and T. Iwaki (2004)
J. Virol. 78, 1281-1288
   Abstract »    Full Text »    PDF »
Strain-specific Kinetics of Prion Protein Formation in Vitro and in Vivo.
E. R. Mulcahy and R. A. Bessen (2004)
J. Biol. Chem. 279, 1643-1649
   Abstract »    Full Text »    PDF »
Reduced proteinase K resistance and infectivity of prions after pressure treatment at 60 {degrees}C.
A. Fernandez Garcia, P. Heindl, H. Voigt, M. Buttner, D. Wienhold, P. Butz, J. Starke, B. Tauscher, and E. Pfaff (2004)
J. Gen. Virol. 85, 261-264
   Abstract »    Full Text »    PDF »
Delay in onset of prion disease for the HY strain of transmissible mink encephalopathy as a result of prior peripheral inoculation with the replication-deficient DY strain.
J. C. Bartz, J. M. Aiken, and R. A. Bessen (2004)
J. Gen. Virol. 85, 265-273
   Abstract »    Full Text »    PDF »
Structural Properties of Gerstmann-Straussler-Scheinker Disease Amyloid Protein.
M. Salmona, M. Morbin, T. Massignan, L. Colombo, G. Mazzoleni, R. Capobianco, L. Diomede, F. Thaler, L. Mollica, G. Musco, et al. (2003)
J. Biol. Chem. 278, 48146-48153
   Abstract »    Full Text »    PDF »
Formation of Critical Oligomers Is a Key Event during Conformational Transition of Recombinant Syrian Hamster Prion Protein.
F. Sokolowski, A. J. Modler, R. Masuch, D. Zirwer, M. Baier, G. Lutsch, D. A. Moss, K. Gast, and D. Naumann (2003)
J. Biol. Chem. 278, 40481-40492
   Abstract »    Full Text »    PDF »
A quantitative, highly sensitive cell-based infectivity assay for mouse scrapie prions.
P.-C. Klohn, L. Stoltze, E. Flechsig, M. Enari, and C. Weissmann (2003)
PNAS 100, 11666-11671
   Abstract »    Full Text »    PDF »
Modulation of prion protein structural integrity by geldanamycin.
H.-J. Ochel, G. Gademann, J. Trepel, and L. Neckers (2003)
Glycobiology 13, 655-660
   Abstract »    Full Text »    PDF »
Evaluation of Quinacrine Treatment for Prion Diseases.
A. Barret, F. Tagliavini, G. Forloni, C. Bate, M. Salmona, L. Colombo, A. De Luigi, L. Limido, S. Suardi, G. Rossi, et al. (2003)
J. Virol. 77, 8462-8469
   Abstract »    Full Text »    PDF »
In vitro cell-free conversion of bacterial recombinant PrP to PrPres as a model for conversion.
L. Kirby, C. R. Birkett, H. Rudyk, I. H. Gilbert, and J. Hope (2003)
J. Gen. Virol. 84, 1013-1020
   Abstract »    Full Text »    PDF »
One O-linked sugar can affect the coil-to-beta structural transition of the prion peptide.
P.-Y. Chen, C.-C. Lin, Y.-T. Chang, S.-C. Lin, and S. I. Chan (2002)
PNAS 99, 12633-12638
   Abstract »    Full Text »    PDF »
Unhampered Prion Neuroinvasion despite Impaired Fast Axonal Transport in Transgenic Mice Overexpressing Four-Repeat Tau.
V. Kunzi, M. Glatzel, M. Y. Nakano, U. F. Greber, F. Van Leuven, and A. Aguzzi (2002)
J. Neurosci. 22, 7471-7477
   Abstract »    Full Text »    PDF »
Induction of Protein Conformational Change in Mouse Senile Amyloidosis.
Y. Xing, A. Nakamura, T. Korenaga, Z. Guo, J. Yao, X. Fu, T. Matsushita, K. Kogishi, M. Hosokawa, F. Kametani, et al. (2002)
J. Biol. Chem. 277, 33164-33169
   Abstract »    Full Text »    PDF »
Presenile dementia syndromes: an update on taxonomy and diagnosis.
M D Greicius, M D Geschwind, and B L Miller (2002)
J. Neurol. Neurosurg. Psychiatry 72, 691-700
   Abstract »    Full Text »    PDF »
Characterization of Thermodynamic Diversity between Transmissible Spongiform Encephalopathy Agent Strains and Its Theoretical Implications.
R. A. Somerville, R. C. Oberthur, U. Havekost, F. MacDonald, D. M. Taylor, and A. G. Dickinson (2002)
J. Biol. Chem. 277, 11084-11089
   Abstract »    Full Text »    PDF »
Prion protein gene polymorphisms in natural goat scrapie.
C. Billinis, C. H. Panagiotidis, V. Psychas, S. Argyroudis, A. Nicolaou, S. Leontides, O. Papadopoulos, and T. Sklaviadis (2002)
J. Gen. Virol. 83, 713-721
   Abstract »    Full Text »    PDF »
PrP Expression and Replication by Schwann Cells: Implications in Prion Spreading.
J. Follet, C. Lemaire-Vieille, F. Blanquet-Grossard, V. Podevin-Dimster, S. Lehmann, J.-P. Chauvin, J.-P. Decavel, R. Varea, J. Grassi, M. Fontes, et al. (2002)
J. Virol. 76, 2434-2439
   Abstract »    Full Text »    PDF »
Use of a Marker Organism To Model the Spread of Central Nervous System Tissue in Cattle and the Abattoir Environment during Commercial Stunning and Carcass Dressing.
D. J. Daly, D. M. Prendergast, J. J. Sheridan, I. S. Blair, and D. A. McDowell (2002)
Appl. Envir. Microbiol. 68, 791-798
   Abstract »    Full Text »    PDF »
The Use of Antioxidants in Transmissible Spongiform Encephalopathies: A Case Report.
J. A. Drisko (2002)
J. Am. Coll. Nutr. 21, 22-25
   Abstract »    Full Text »    PDF »
A Critical Review of the Nature of the Spongiform Encephalopathy Agent: Protein Theory Versus Virus Theory.
H. Narang (2002)
Experimental Biology and Medicine 227, 4-19
   Abstract »    Full Text »    PDF »
Sheep Prion Protein Synthetic Peptide Spanning Helix 1 and beta -Strand 2 (Residues 142-166) Shows beta -Hairpin Structure in Solution.
S. A. Kozin, G. Bertho, A. K. Mazur, H. Rabesona, J.-P. Girault, T. Haertle, M. Takahashi, P. Debey, and G. H. B. Hoa (2001)
J. Biol. Chem. 276, 46364-46370
   Abstract »    Full Text »    PDF »
Prion Protein Fragment PrP-(106-126) Induces Apoptosis via Mitochondrial Disruption in Human Neuronal SH-SY5Y Cells.
C. N. O'Donovan, D. Tobin, and T. G. Cotter (2001)
J. Biol. Chem. 276, 43516-43523
   Abstract »    Full Text »    PDF »
Deletion of {beta}-Strand and {alpha}-Helix Secondary Structure in Normal Prion Protein Inhibits Formation of Its Protease-Resistant Isoform.
I. Vorberg, K. Chan, and S. A. Priola (2001)
J. Virol. 75, 10024-10032
   Abstract »    Full Text »    PDF »
A Critical Review of Atypical Cerebellum-Type Creutzfeldt-Jakob Disease: Its Relationship to ``New Variant'' CJD and Bovine Spongiform Encephalopathy.
H. K. Narang (2001)
Experimental Biology and Medicine 226, 629-639
   Abstract »    Full Text »    PDF »
Neurodegenerative Diseases and Prions.
S. B. Prusiner (2001)
N. Engl. J. Med. 344, 1516-1526
   Full Text »    PDF »
PoPMuSiC, an algorithm for predicting protein mutant stability changes. Application to prion proteins.
D. Gilis and M. Rooman (2000)
Protein Eng. Des. Sel. 13, 849-856
   Abstract »    Full Text »    PDF »
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 Degeneration.
A. Li, S. Sakaguchi, K. Shigematsu, R. Atarashi, B. C. Roy, R. Nakaoke, K. Arima, N. Okimura, J. Kopacek, and S. Katamine (2000)
Am. J. Pathol. 157, 1447-1452
   Abstract »    Full Text »    PDF »
Strain-specific propagation of PrPSc properties into baculovirus-expressed hamster PrPC.
V. Iniguez, D. McKenzie, J. Mirwald, and J. Aiken (2000)
J. Gen. Virol. 81, 2565-2571
   Abstract »    Full Text »
Molecular dynamics simulation of human prion protein including both N-linked oligosaccharides and the GPI anchor.
J. Zuegg and J. E. Gready (2000)
Glycobiology 10, 959-974
   Abstract »    Full Text »    PDF »
Follicular Dendritic Cells and Dissemination of Creutzfeldt-Jakob Disease.
L. Manuelidis, I. Zaitsev, P. Koni, Z. Yun Lu, R. A. Flavell, and W. Fritch (2000)
J. Virol. 74, 8614-8622
   Abstract »    Full Text »
Creutzfeldt-Jakob disease with a novel four extra-repeat insertional mutation in the PrP gene.
G. Rossi, G. Giaccone, L. Giampaolo, S. Iussich, G. Puoti, M. Frigo, G. Cavaletti, L. Frattola, O. Bugiani, and F. Tagliavini (2000)
Neurology 55, 405-410
   Abstract »    Full Text »    PDF »
Adaptation and Selection of Prion Protein Strain Conformations following Interspecies Transmission of Transmissible Mink Encephalopathy.
J. C. Bartz, R. A. Bessen, D. McKenzie, R. F. Marsh, and J. M. Aiken (2000)
J. Virol. 74, 5542-5547
   Abstract »    Full Text »
Dominant-Negative Inhibition of Prion Formation Diminished by Deletion Mutagenesis of the Prion Protein.
L. Zulianello, K. Kaneko, M. Scott, S. Erpel, D. Han, F. E. Cohen, and S. B. Prusiner (2000)
J. Virol. 74, 4351-4360
   Abstract »    Full Text »
Successful Transmission of Three Mouse-Adapted Scrapie Strains to Murine Neuroblastoma Cell Lines Overexpressing Wild-Type Mouse Prion Protein.
N. Nishida, D. A. Harris, D. Vilette, H. Laude, Y. Frobert, J. Grassi, D. Casanova, O. Milhavet, and S. Lehmann (2000)
J. Virol. 74, 320-325
   Abstract »    Full Text »
Upregulation of the Genes Encoding Lysosomal Hydrolases, a Perforin-Like Protein, and Peroxidases in the Brains of Mice Affected with an Experimental Prion Disease.
J. Kopacek, S. Sakaguchi, K. Shigematsu, N. Nishida, R. Atarashi, R. Nakaoke, R. Moriuchi, M. Niwa, and S. Katamine (2000)
J. Virol. 74, 411-417
   Abstract »    Full Text »
Glycosylation differences between the normal and pathogenic prion protein isoforms.
P. M. Rudd, T. Endo, C. Colominas, D. Groth, S. F. Wheeler, D. J. Harvey, M. R. Wormald, H. Serban, S. B. Prusiner, A. Kobata, et al. (1999)
PNAS 96, 13044-13049
   Abstract »    Full Text »    PDF »
PrP gene polymorphism and natural scrapie in Icelandic sheep.
S. Thorgeirsdottir, S. Sigurdarson, H. M. Thorisson, G. Georgsson, and A. Palsdottir (1999)
J. Gen. Virol. 80, 2527-2534
   Abstract »    Full Text »
Folding funnels and binding mechanisms.
B. Ma, S. Kumar, C.-J. Tsai, and R. Nussinov (1999)
Protein Eng. Des. Sel. 12, 713-720
   Abstract »    Full Text »    PDF »
Sphingolipid Depletion Increases Formation of the Scrapie Prion Protein in Neuroblastoma Cells Infected with Prions.
N. Naslavsky, H. Shmeeda, G. Friedlander, A. Yanai, A. H. Futerman, Y. Barenholz, and A. Taraboulos (1999)
J. Biol. Chem. 274, 20763-20771
   Abstract »    Full Text »    PDF »
A Delicate Balance: Homeostatic Control of Copper Uptake and Distribution.
M. M. O. Peña, J. Lee, and D. J. Thiele (1999)
J. Nutr. 129, 1251-1260
   Abstract »    Full Text »
Effectiveness of Polyene Antibiotics in Treatment of Transmissible Spongiform Encephalopathy in Transgenic Mice Expressing Syrian Hamster PrP Only in Neurons.
R. Demaimay, R. Race, and B. Chesebro (1999)
J. Virol. 73, 3511-3513
   Abstract »    Full Text »
Reversible Conversion of Monomeric Human Prion Protein Between Native and Fibrilogenic Conformations.
G. S. Jackson, L. L. Hosszu, A. Power, A. F. Hill, J. Kenney, H. Saibil, C. J. Craven, J. P. Waltho, A. R. Clarke, and J. Collinge (1999)
Science 283, 1935-1937
   Abstract »    Full Text »
Molecular Genetics of Transmissible Spongiform Encephalopathies.
C. Weissmann (1999)
J. Biol. Chem. 274, 3-6
   Full Text »    PDF »
Expression of Cellular Prion Protein in Activated Hepatic Stellate Cells.
K. Ikeda, N. Kawada, Y. Q. Wang, H. Kadoya, K. Nakatani, M. Sato, and K. Kaneda (1998)
Am. J. Pathol. 153, 1695-1700
   Abstract »    Full Text »    PDF »



To Advertise     Find Products


Science. ISSN 0036-8075 (print), 1095-9203 (online)