Previous Article |
Table of Contents
| Next Article 
Vol. 96, Issue 26, 15137-15142, December 21, 1999
* Institute for Neurodegenerative Diseases, Departments of
Contributed by Stanley B. Prusiner, October 27, 1999
There is growing concern that bovine spongiform encephalopathy
(BSE) may have passed from cattle to humans. We report here that
transgenic (Tg) mice expressing bovine (Bo) prion protein (PrP)
serially propagate BSE prions and that there is no species barrier for
transmission from cattle to Tg(BoPrP) mice. These same mice were also
highly susceptible to a new variant of Creutzfeldt-Jakob disease
(nvCJD) and natural sheep scrapie. The incubation times (
Medical Sciences
Compelling transgenetic evidence for transmission of bovine
spongiform encephalopathy prions to humans
,
,,,¶, and,
Neurology, Biochemistry and Biophysics,
and ¶ Pathology, University of California, San
Francisco, CA 94143; and § National CJD Surveillance Unit,
Western General Hospital, Edinburgh EH4 2XU, United Kingdom
250 days),
neuropathology, and disease-causing PrP isoforms in
Tg(BoPrP)Prnp0/0 mice inoculated with nvCJD and BSE brain
extracts were indistinguishable and differed dramatically from those
seen in these mice injected with natural scrapie prions. Our findings
provide the most compelling evidence to date that prions from cattle
with BSE have infected humans and caused fatal neurodegeneration.
To whom reprint requests should be addressed at:
Institute for Neurodegenerative Diseases, Department of Neurology, Box
0518, University of California, San Francisco, CA 94143-0518. E-mail: abbott{at}itsa.ucsf.edu.
Copyright © 1999 by The National Academy of Sciences 0027-8424/99/9615137-6$2.00/0
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg What's this?
This article has been cited by other articles in HighWire Press-hosted journals:
|
|
 |
|
  M. D. Zabel, M. Heikenwalder, M. Prinz, I. Arrighi, P. Schwarz, J. Kranich, A. von Teichman, K. M. Haas, N. Zeller, T. F. Tedder, et al. Stromal Complement Receptor CD21/35 Facilitates Lymphoid Prion Colonization and Pathogenesis J. Immunol., November 1, 2007; 179(9): 6144 - 6152. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. Benatar The Chickens Come Home to Roost Am J Public Health, September 1, 2007; 97(9): 1545 - 1546. [Full Text] [PDF]
|
|
|
|
 |
|
 V. Beringue, O. Andreoletti, A. Le Dur, R. Essalmani, J.-L. Vilotte, C. Lacroux, F. Reine, L. Herzog, A.-G. Biacabe, T. Baron, et al. A Bovine Prion Acquires an Epidemic Bovine Spongiform Encephalopathy Strain-Like Phenotype on Interspecies Transmission J. Neurosci., June 27, 2007; 27(26): 6965 - 6971. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 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. Sheep-Passaged Bovine Spongiform Encephalopathy Agent Exhibits Altered Pathobiological Properties in Bovine-PrP Transgenic Mice J. Virol., January 15, 2007; 81(2): 835 - 843. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M W Head and J W Ironside vCJD and the gut: implications for endoscopy Gut, January 1, 2007; 56(1): 9 - 11. [Full Text] [PDF]
|
|
|
|
 |
|
 C. Cordier, A. Bencsik, S. Philippe, D. Betemps, F. Ronzon, D. Calavas, C. Crozet, and T. Baron Transmission and characterization of bovine spongiform encephalopathy sources in two ovine transgenic mouse lines (TgOvPrP4 and TgOvPrP59) J. Gen. Virol., December 1, 2006; 87(12): 3763 - 3771. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 X. WU, Z. WANG, Y. LIU, Y. ZHANG, Y. CHEN, and Y. ZOU Development of a Monoclonal Antibody-Based Immunohistochemistry Method for BSE Surveillance in China Ann. N.Y. Acad. Sci., October 1, 2006; 1081(1): 112 - 123. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Lezmi, A. Bencsik, and T. Baron PET-blot Analysis Contributes to BSE Strain Recognition in C57Bl/6 Mice J. Histochem. Cytochem., October 1, 2006; 54(10): 1087 - 1094. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. Tamguney, K. Giles, E. Bouzamondo-Bernstein, P. J. Bosque, M. W. Miller, J. Safar, S. J. DeArmond, and S. B. Prusiner Transmission of elk and deer prions to transgenic mice. J. Virol., September 1, 2006; 80(18): 9104 - 9114. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. J. Trifilo, T. Yajima, Y. Gu, N. Dalton, K. L. Peterson, R. E. Race, K. Meade-White, J. L. Portis, E. Masliah, K. U. Knowlton, et al. Prion-induced amyloid heart disease with high blood infectivity in transgenic mice. Science, July 7, 2006; 313(5783): 94 - 97. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 E. M. Jones, K. Surewicz, and W. K. Surewicz Role of N-terminal Familial Mutations in Prion Protein Fibrillization and Prion Amyloid Propagation in Vitro J. Biol. Chem., March 24, 2006; 281(12): 8190 - 8196. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Z. Xie, K. I. O'Rourke, Z. Dong, A. L. Jenny, J. A. Langenberg, E. D. Belay, L. B. Schonberger, R. B. Petersen, W. Zou, Q. Kong, et al. Chronic Wasting Disease of Elk and Deer and Creutzfeldt-Jakob Disease: COMPARATIVE ANALYSIS OF THE SCRAPIE PRION PROTEIN J. Biol. Chem., February 17, 2006; 281(7): 4199 - 4206. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
O. Windl, M. Buchholz, A. Neubauer, W. Schulz-Schaeffer, M. Groschup, S. Walter, S. Arendt, M. Neumann, A. K. Voss, and H. A. Kretzschmar Breaking an Absolute Species Barrier: Transgenic Mice Expressing the Mink PrP Gene Are Susceptible to Transmissible Mink Encephalopathy J. Virol., December 1, 2005; 79(23): 14971 - 14975. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Nunziante, C. Kehler, E. Maas, M. U. Kassack, M. Groschup, and H. M. Schatzl Charged bipolar suramin derivatives induce aggregation of the prion protein at the cell surface and inhibit PrPSc replication J. Cell Sci., November 1, 2005; 118(21): 4959 - 4973. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. G. Safar, S. J. DeArmond, K. Kociuba, C. Deering, S. Didorenko, E. Bouzamondo-Bernstein, S. B. Prusiner, and P. Tremblay Prion clearance in bigenic mice J. Gen. Virol., October 1, 2005; 86(10): 2913 - 2923. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Q. Kong, S. Huang, W. Zou, D. Vanegas, M. Wang, D. Wu, J. Yuan, M. Zheng, H. Bai, H. Deng, et al. Chronic Wasting Disease of Elk: Transmissibility to Humans Examined by Transgenic Mouse Models J. Neurosci., August 31, 2005; 25(35): 7944 - 7949. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. De Simone, G. G. Dodson, C. S. Verma, A. Zagari, and F. Fraternali Prion and water: Tight and dynamical hydration sites have a key role in structural stability PNAS, May 24, 2005; 102(21): 7535 - 7540. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. Ladogana, M. Puopolo, E. A. Croes, H. Budka, C. Jarius, S. Collins, G. M. Klug, T. Sutcliffe, A. Giulivi, A. Alperovitch, et al. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada Neurology, May 10, 2005; 64(9): 1586 - 1591. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. R. Scott, D. Peretz, H.-O. B. Nguyen, S. J. DeArmond, and S. B. Prusiner Transmission Barriers for Bovine, Ovine, and Human Prions in Transgenic Mice J. Virol., May 1, 2005; 79(9): 5259 - 5271. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. Gonzalez, S. Martin, F. E. Houston, N. Hunter, H. W. Reid, S. J. Bellworthy, and M. Jeffrey Phenotype of disease-associated PrP accumulation in the brain of bovine spongiform encephalopathy experimentally infected sheep J. Gen. Virol., March 1, 2005; 86(3): 827 - 838. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. Saegerman, D. Berkvens, L. Claes, A. Dewaele, F. Coignoul, R. Ducatelle, D. Cassart, B. Brochier, F. Costy, S. Roels, et al. Population-Level Retrospective Study of Neurologically Expressed Disorders in Ruminants before the Onset of Bovine Spongiform Encephalopathy (BSE) in Belgium, a BSE Risk III Country J. Clin. Microbiol., February 1, 2005; 43(2): 862 - 869. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. Lemmer, M. Mielke, G. Pauli, and M. Beekes Decontamination of surgical instruments from prion proteins: in vitro studies on the detachment, destabilization and degradation of PrPSc bound to steel surfaces J. Gen. Virol., December 1, 2004; 85(12): 3805 - 3816. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Serban, G. Legname, K. Hansen, N. Kovaleva, and S. B. Prusiner Immunoglobulins in Urine of Hamsters with Scrapie J. Biol. Chem., November 19, 2004; 279(47): 48817 - 48820. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Thomzig, S. Spassov, M. Friedrich, D. Naumann, and M. Beekes Discriminating Scrapie and Bovine Spongiform Encephalopathy Isolates by Infrared Spectroscopy of Pathological Prion Protein J. Biol. Chem., August 6, 2004; 279(32): 33847 - 33854. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. Baron, C. Crozet, A.-G. Biacabe, S. Philippe, J. Verchere, A. Bencsik, J.-Y. Madec, D. Calavas, and J. Samarut Molecular Analysis of the Protease-Resistant Prion Protein in Scrapie and Bovine Spongiform Encephalopathy Transmitted to Ovine Transgenic and Wild-Type Mice J. Virol., June 15, 2004; 78(12): 6243 - 6251. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
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. Subclinical Bovine Spongiform Encephalopathy Infection in Transgenic Mice Expressing Porcine Prion Protein J. Neurosci., May 26, 2004; 24(21): 5063 - 5069. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Casalone, G. Zanusso, P. Acutis, S. Ferrari, L. Capucci, F. Tagliavini, S. Monaco, and M. Caramelli Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease PNAS, March 2, 2004; 101(9): 3065 - 3070. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. Tremblay, H. L. Ball, K. Kaneko, D. Groth, R. S. Hegde, F. E. Cohen, S. J. DeArmond, S. B. Prusiner, and J. G. Safar Mutant PrPSc Conformers Induced by a Synthetic Peptide and Several Prion Strains J. Virol., February 15, 2004; 78(4): 2088 - 2099. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. W. Head, D. Ritchie, N. Smith, V. McLoughlin, W. Nailon, S. Samad, S. Masson, M. Bishop, L. McCardle, and J. W. Ironside Peripheral Tissue Involvement in Sporadic, Iatrogenic, and Variant Creutzfeldt-Jakob Disease: An Immunohistochemical, Quantitative, and Biochemical Study Am. J. Pathol., January 1, 2004; 164(1): 143 - 153. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. L. Mobley, D. L. Cox, R. R. P. Singh, R. V. Kulkarni, and A. Slepoy Simulations of Oligomeric Intermediates in Prion Diseases Biophys. J., October 1, 2003; 85(4): 2213 - 2223. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Bresjanac, L. M. Smid, T. D. Vovko, A. Petric, J. R. Barrio, and M. Popovic Molecular-Imaging Probe 2-(1-{6-[(2-Fluoroethyl)(Methyl) Amino]-2-Naphthyl}Ethylidene) Malononitrile Labels Prion Plaques In Vitro J. Neurosci., September 3, 2003; 23(22): 8029 - 8033. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. A. Collie, D. M. Summers, R. J. Sellar, J. W. Ironside, S. Cooper, M. Zeidler, R. Knight, and R. G. Will Diagnosing Variant Creutzfeldt-Jakob Disease with the Pulvinar Sign: MR Imaging Findings in 86 Neuropathologically Confirmed Cases AJNR Am. J. Neuroradiol., September 1, 2003; 24(8): 1560 - 1569. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C Weissmann and E Flechsig PrP knock-out and PrP transgenic mice in prion research Br. Med. Bull., June 1, 2003; 66(1): 43 - 60. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. G Will Acquired prion disease: iatrogenic CJD, variant CJD, kuru Br. Med. Bull., June 1, 2003; 66(1): 255 - 265. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. Weitkunat, C. Pottgiesser, N. Meyer, A. Crispin, R. Fischer, K. Schotten, J. Kerr, and K. Uberla Perceived Risk of Bovine Spongiform Encephalopathy and Dietary Behavior J Health Psychol, May 1, 2003; 8(3): 373 - 381. [Abstract] [PDF]
|
|
|
|
|
|
C. Korth, K. Kaneko, D. Groth, N. Heye, G. Telling, J. Mastrianni, P. Parchi, P. Gambetti, R. Will, J. Ironside, et al. Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene PNAS, April 15, 2003; 100(8): 4784 - 4789. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. C. H. May, A. T. Fafarman, S. B. Hong, M. Rogers, L. W. Deady, S. B. Prusiner, and F. E. Cohen Potent inhibition of scrapie prion replication in cultured cells by bis-acridines PNAS, March 18, 2003; 100(6): 3416 - 3421. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. D. Belay, R. A. Maddox, P. Gambetti, and L. B. Schonberger Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States Neurology, January 28, 2003; 60(2): 176 - 181. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. J.T. Ward, D. Everington, E. A. Croes, A. Alperovitch, N. Delasnerie-Laupretre, I. Zerr, S. Poser, and C. M. van Duijn Sporadic Creutzfeldt-Jakob disease and surgery: A case-control study using community controls Neurology, August 27, 2002; 59(4): 543 - 548. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M D Greicius, M D Geschwind, and B L Miller Presenile dementia syndromes: an update on taxonomy and diagnosis J. Neurol. Neurosurg. Psychiatry, June 1, 2002; 72(6): 691 - 700. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. J. Bosque, C. Ryou, G. Telling, D. Peretz, G. Legname, S. J. DeArmond, and S. B. Prusiner Prions in skeletal muscle PNAS, March 19, 2002; 99(6): 3812 - 3817. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C E M Hillier, R L Salmon, J W Neal, and D A Hilton Possible underascertainment of variant Creutzfeldt-Jakob disease: a systematic study J. Neurol. Neurosurg. Psychiatry, March 1, 2002; 72(3): 304 - 309. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. A Venters New variant Creutzfeldt-Jakob disease: the epidemic that never was BMJ, October 13, 2001; 323(7317): 858 - 861. [Full Text] [PDF]
|
|
|
|
|
|
C. Korth, B. C. H. May, F. E. Cohen, and S. B. Prusiner Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease PNAS, August 14, 2001; 98(17): 9836 - 9841. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. B. Coulthart and N. R. Cashman Variant Creutzfeldt-Jakob disease: a summary of current scientific knowledge in relation to public health Can. Med. Assoc. J., July 1, 2001; 165(1): 51 - 58. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J.-L. Vilotte, S. Soulier, R. Essalmani, M.-G. Stinnakre, D. Vaiman, L. Lepourry, J. C. Da Silva, N. Besnard, M. Dawson, A. Buschmann, et al. Markedly Increased Susceptibility to Natural Sheep Scrapie of Transgenic Mice Expressing Ovine PrP J. Virol., July 1, 2001; 75(13): 5977 - 5984. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Crozet, F. Flamant, A. Bencsik, D. Aubert, J. Samarut, and T. Baron Efficient Transmission of Two Different Sheep Scrapie Isolates in Transgenic Mice Expressing the Ovine PrP Gene J. Virol., June 1, 2001; 75(11): 5328 - 5334. [Abstract] [Full Text]
|
|
|
|
 |
|
 S. B. Prusiner Neurodegenerative Diseases and Prions N. Engl. J. Med., May 17, 2001; 344(20): 1516 - 1526. [Full Text] [PDF]
|
|
|
|
|
|
S. Supattapone, H. Wille, L. Uyechi, J. Safar, P. Tremblay, F. C. Szoka, F. E. Cohen, S. B. Prusiner, and M. R. Scott Branched Polyamines Cure Prion-Infected Neuroblastoma Cells J. Virol., April 1, 2001; 75(7): 3453 - 3461. [Abstract] [Full Text]
|
|
|
|
|
|
C. I. Lasmézas, J.-G. Fournier, V. Nouvel, H. Boe, D. Marcé, F. Lamoury, N. Kopp, J.-J. Hauw, J. Ironside, M. Bruce, et al. Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt- Jakob disease: Implications for human health PNAS, March 16, 2001; (2001) 41490898. [Abstract] [Full Text]
|
|
|
|
|
|
T. R. Appel, M. Wolff, F. von Rheinbaben, M. Heinzel, and D. Riesner Heat stability of prion rods and recombinant prion protein in water, lipid and lipid-water mixtures J. Gen. Virol., February 1, 2001; 82(2): 465 - 473. [Abstract] [Full Text]
|
|
|
|
|
|
M. Glatzel and A. Aguzzi PrPC expression in the peripheral nervous system is a determinant of prion neuroinvasion J. Gen. Virol., November 1, 2000; 81(11): 2813 - 2821. [Abstract] [Full Text]
|
|
|
|
|
|
P. Brown, M. Preece, J.-P. Brandel, T. Sato, L. McShane, I. Zerr, A. Fletcher, R. G. Will, M. Pocchiari, N. R. Cashman, et al. Iatrogenic Creutzfeldt-Jakob disease at the millennium Neurology, October 24, 2000; 55(8): 1075 - 1081. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. E. M. Hillier and R. L. Salmon Is there evidence for exogenous risk factors in the aetiology and spread of Creutzfeldt-Jakob disease? QJM, September 1, 2000; 93(9): 617 - 631. [Full Text] [PDF]
|
|
|
|
|
|
S. A. Norton Raw Animal Tissues and Dietary Supplements N. Engl. J. Med., July 27, 2000; 343(4): 304 - 305. [Full Text]
|
|
|
|
|
