Stanley B. Prusiner

Stanley Ben Prusiner is an American neurologist and biochemist. Prusiner discovered a type of infectious self-reproducing pathogens which are composed of proteins. He named this class as “Prions”. For his research work Prusiner was awarded with Nobel Prize in Physiology or Medicine 1997. He had also received the Albert Lasker Award for Basic Medical Research 1994 for the same work.

Research findings made by Stanley Ben Prusiner made a new way in the research of diseases caused by prions such as bovine spongiform encephalopathy (BSE) or “mad cow disease”. This research is also helpful for Alzheimer’s disease and Parkinson’s disease which share similar characteristics as the prion diseases.

Stanley Ben Prusiner also received the Dickson Prize 1992 and Wolf Prize in Medicine 1996.

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1991 Potamkin Prize for Alzheimer’s Disease Research

1992 Christopher Columbus Quincentennial Discovery Award in Biomedical Research

1992 Metropolitan Life Foundation Award for Medical Research

1992 Dickson Prize for Distinguished Scientific Accomplishments, University of Pittsburgh

1992 Charles A. Dana Award for Pioneering Achievements in Health

1993 Richard Lounsbery Award for Extraordinary Scientific Research

1993 Gairdner Foundation Award for Outstanding Achievement in Medical Science

1994 Bristol-Myers Squibb Award for Distinguished Achievement in Neuroscience Res.

1994 Albert Lasker Award for Basic Medical Research, Albert and Mary Lasker Foundation

1995 Paul Ehrlich Prize, Paul Ehrlich Foundation and the Federal Republic of Germany

1996 Wolf Prize in Medicine, Wolf Foundation and the State of Israel

1996 Keio International Award for Medical Science, Keio University, Tokyo, Japan

1997 Nobel Prize in Physiology or Medicine

Alejandra Gallardo-Godoy; Joel Gever; Kimberly L Fife; B Michael Silber; Stanley B Prusiner; Adam R Renslo 2-Aminothiazoles as therapeutic leads for prion diseases. Journal of medicinal chemistry 2011;54(4):1010-21.

Joel C Watts; Kurt Giles; Sunny K Grillo; Azucena Lemus; Stephen J DeArmond; Stanley B Prusiner Bioluminescence imaging of Abeta deposition in bigenic mouse models of Alzheimer’s disease. Proceedings of the National Academy of Sciences of the United States of America 2011;108(6):2528-33.

David W Colby; Stanley B Prusiner Prions. Cold Spring Harbor perspectives in biology 2011;3(1):a006833.

John C Price; Shenheng Guan; Alma Burlingame; Stanley B Prusiner; Sina Ghaemmaghami Analysis of proteome dynamics in the mouse brain. Proceedings of the National Academy of Sciences of the United States of America 2010;107(32):14508-13.

Kurt Giles; David V Glidden; Smita Patel; Carsten Korth; Darlene Groth; Azucena Lemus; Stephen J DeArmond; Stanley B Prusiner Human prion strain selection in transgenic mice. Annals of neurology 2010;68(2):151-61.

Larry H Stanker; Ana V Serban; Elisa Cleveland; Robert Hnasko; Azucena Lemus; Jiri Safar; Stephen J DeArmond; Stanley B Prusiner Conformation-dependent high-affinity monoclonal antibodies to prion proteins. Journal of immunology (Baltimore, Md. : 1950) 2010;185(1):729-37.

Allison K Doak; Holger Wille; Stanley B Prusiner; Brian K Shoichet Colloid formation by drugs in simulated intestinal fluid. Journal of medicinal chemistry 2010;53(10):4259-65.

Sina Ghaemmaghami; Julie Ullman; Misol Ahn; Susan St Martin; Stanley B Prusiner Chemical induction of misfolded prion protein conformers in cell culture. The Journal of biological chemistry 2010;285(14):10415-23.

Sina Ghaemmaghami; Barnaby C H May; Adam R Renslo; Stanley B Prusiner Discovery of 2-aminothiazoles as potent antiprion compounds. Journal of virology 2010;84(7):3408-12.

Robert Hnasko; Ana V Serban; George Carlson; Stanley B Prusiner; Larry H Stanker Generation of antisera to purified prions in lipid rafts. Prion 2010;4(2):94-104.

David W Colby; Rachel Wain; Ilia V Baskakov; Giuseppe Legname; Christina G Palmer; Hoang-Oanh B Nguyen; Azucena Lemus; Fred E Cohen; Stephen J DeArmond; Stanley B Prusiner Protease-sensitive synthetic prions. PLoS pathogens 2010;6(1):e1000736.

David W Colby; Kurt Giles; Giuseppe Legname; Holger Wille; Ilia V Baskakov; Stephen J DeArmond; Stanley B Prusiner Design and construction of diverse mammalian prion strains. Proceedings of the National Academy of Sciences of the United States of America 2009;106(48):20417-22.

Sina Ghaemmaghami; Misol Ahn; Pierre Lessard; Kurt Giles; Giuseppe Legname; Stephen J DeArmond; Stanley B Prusiner Continuous quinacrine treatment results in the formation of drug-resistant prions. PLoS pathogens 2009;5(11):e1000673.

Holger Wille; Wen Bian; Michele McDonald; Amy Kendall; David W Colby; Lillian Bloch; Julian Ollesch; Alexander L Borovinskiy; Fred E Cohen; Stanley B Prusiner; et al. Natural and synthetic prion structure from X-ray fiber diffraction. Proceedings of the National Academy of Sciences of the United States of America 2009;106(40):16990-5.

Gültekin Tamgüney; Michael W Miller; Lisa L Wolfe; Tracey M Sirochman; David V Glidden; Christina Palmer; Azucena Lemus; Stephen J DeArmond; Stanley B Prusiner Asymptomatic deer excrete infectious prions in faeces. Nature 2009;461(7263):529-32.

Gültekin Tamgüney; Kevin P Francis; Kurt Giles; Azucena Lemus; Stephen J DeArmond; Stanley B Prusiner Measuring prions by bioluminescence imaging. Proceedings of the National Academy of Sciences of the United States of America 2009;106(35):15002-6.

Sung-Hun Bae; Giuseppe Legname; Ana Serban; Stanley B Prusiner; Peter E Wright; H Jane Dyson Prion proteins with pathogenic and protective mutations show similar structure and dynamics. Biochemistry 2009;48(34):8120-8.

C Warren Olanow; Stanley B Prusiner Is Parkinson’s disease a prion disorder? Proceedings of the National Academy of Sciences of the United States of America 2009;106(31):12571-2.

Ed M Choi; Michael D Geschwind; Camille Deering; Kristen Pomeroy; Amy Kuo; Bruce L Miller; Jiri G Safar; Stanley B Prusiner Prion proteins in subpopulations of white blood cells from patients with sporadic Creutzfeldt-Jakob disease. Laboratory investigation; a journal of technical methods and pathology 2009;89(6):624-35.

Gültekin Tamgüney; Michael W Miller; Kurt Giles; Azucena Lemus; David V Glidden; Stephen J DeArmond; Stanley B Prusiner Transmission of scrapie and sheep-passaged bovine spongiform encephalopathy prions to transgenic mice expressing elk prion protein. The Journal of general virology 2009;90(Pt 4):1035-47.

Vincenza Campana; Lorena Zentilin; Ilaria Mirabile; Agata Kranjc; Philippe Casanova; Mauro Giacca; Stanley B Prusiner; Giuseppe Legname; Chiara Zurzolo Development of antibody fragments for immunotherapy of prion diseases. The Biochemical journal 2009;418(3):507-15.

Holger Wille; Maheswaran Shanmugam; Muralee Murugesu; Julian Ollesch; Gerald Stubbs; Jeffrey R Long; Jiri G Safar; Stanley B Prusiner Surface charge of polyoxometalates modulates polymerization of the scrapie prion protein. Proceedings of the National Academy of Sciences of the United States of America 2009;106(10):3740-5.

Susan F Godsave; Holger Wille; Pekka Kujala; Diane Latawiec; Stephen J DeArmond; Ana Serban; Stanley B Prusiner; Peter J Peters Cryo-immunogold electron microscopy for prions: toward identification of a conversion site. The Journal of neuroscience : the official journal of the Society for Neuroscience 2008;28(47):12489-99.

Kurt Giles; David V Glidden; Robyn Beckwith; Rose Seoanes; David Peretz; Stephen J DeArmond; Stanley B Prusiner Resistance of bovine spongiform encephalopathy (BSE) prions to inactivation. PLoS pathogens 2008;4(11):e1000206.

Patricia Spilman; Pierre Lessard; Mamta Sattavat; Clarissa Bush; Thomas Tousseyn; Eric J Huang; Kurt Giles; Todd Golde; Pritam Das; Abdul Fauq; et al. A gamma-secretase inhibitor and quinacrine reduce prions and prevent dendritic degeneration in murine brains. Proceedings of the National Academy of Sciences of the United States of America 2008;105(30):10595-600.

Gültekin Tamgüney; Kurt Giles; David V Glidden; Pierre Lessard; Holger Wille; Patrick Tremblay; Darlene F Groth; Fruma Yehiely; Carsten Korth; Richard C Moore; et al. Genes contributing to prion pathogenesis. The Journal of general virology 2008;89(Pt 7):1777-88.

Jiri G Safar; Pierre Lessard; Gültekin Tamgüney; Yevgeniy Freyman; Camille Deering; Frederic Letessier; Stephen J Dearmond; Stanley B Prusiner Transmission and detection of prions in feces. The Journal of infectious diseases 2008;198(1):81-9.

Brian Y Feng; Brandon H Toyama; Holger Wille; David W Colby; Sean R Collins; Barnaby C H May; Stanley B Prusiner; Jonathan Weissman; Brian K Shoichet Small-molecule aggregates inhibit amyloid polymerization. Nature chemical biology 2008;4(3):197-9.

Jan Stöhr; Nicole Weinmann; Holger Wille; Tina Kaimann; Luitgard Nagel-Steger; Eva Birkmann; Giannantonio Panza; Stanley B Prusiner; Manfred Eigen; Detlev Riesner Mechanisms of prion protein assembly into amyloid. Proceedings of the National Academy of Sciences of the United States of America 2008;105(7):2409-14.

David W Colby; Qiang Zhang; Shuyi Wang; Darlene Groth; Giuseppe Legname; Detlev Riesner; Stanley B Prusiner Prion detection by an amyloid seeding assay. Proceedings of the National Academy of Sciences of the United States of America 2007;104(52):20914-9.

Henrik Müller; Lothar Stitz; Holger Wille; Stanley B Prusiner; Detlev Riesner Influence of water, fat, and glycerol on the mechanism of thermal prion inactivation. The Journal of biological chemistry 2007;282(49):35855-67.

Holger Wille; Cédric Govaerts; Alexander Borovinskiy; Diane Latawiec; Kenneth H Downing; Fred E Cohen; Stanley B Prusiner Electron crystallography of the scrapie prion protein complexed with heavy metals. Archives of biochemistry and biophysics 2007;467(2):239-48.

Sina Ghaemmaghami; Puay-Wah Phuan; Beth Perkins; Julie Ullman; Barnaby C H May; Fred E Cohen; Stanley B Prusiner Cell division modulates prion accumulation in cultured cells. Proceedings of the National Academy of Sciences of the United States of America 2007;104(46):17971-6.

David J King; Jiri G Safar; Giuseppe Legname; Stanley B Prusiner Thioaptamer interactions with prion proteins: sequence-specific and non-specific binding sites. Journal of molecular biology 2007;369(4):1001-14.

Erling Norrby; Stanley B Prusiner Polio and Nobel prizes: looking back 50 years. Annals of neurology 2007;61(5):385-95.

Margarita Diez; Darlene Groth; Stephen J DeArmond; Stanley B Prusiner; Tomas Hökfelt Changes in neuropeptide expression in mice infected with prions. Neurobiology of aging 2007;28(5):748-65.

Puay-Wah Phuan; Julie A Zorn; Jiri Safar; Kurt Giles; Stanley B Prusiner; Fred E Cohen; Barnaby C H May Discriminating between cellular and misfolded prion protein by using affinity to 9-aminoacridine compounds. The Journal of general virology 2007;88(Pt 4):1392-401.

Patrick Tremblay; Essia Bouzamondo-Bernstein; Cornelia Heinrich; Stanley B Prusiner; Stephen J DeArmond Developmental expression of PrP in the post-implantation embryo. Brain research 2007;1139():60-7.

Wolfgang J Philipp; Darlene Groth; Kurt Giles; Pavel Vodrazka; Heinz Schimmel; Muriel Feyssaguet; Reet Toomik; Pascal Schacher; Awad A Osman; Ingolf Lachmann; et al. Transgenic mouse brains for the evaluation and quality control of BSE tests. Biological chemistry 2007;388(3):349-54.

Marcela V Karpuj; Kurt Giles; Sagit Gelibter-Niv; Michael R Scott; Vishwanath R Lingappa; Francis C Szoka; David Peretz; Wilfred Denetclaw; Stanley B Prusiner Phosphorothioate oligonucleotides reduce PrP levels and prion infectivity in cultured cells. Molecular medicine (Cambridge, Mass.) 2007;13(3-4):190-8.

Barnaby C H May; Julie A Zorn; Juanita Witkop; John Sherrill; Andrew C Wallace; Giuseppe Legname; Stanley B Prusiner; Fred E Cohen Structure-activity relationship study of prion inhibition by 2-aminopyridine-3,5-dicarbonitrile-based compounds: parallel synthesis, bioactivity, and in vitro pharmacokinetics. Journal of medicinal chemistry 2007;50(1):65-73.

Giuseppe Legname; Hoang-Oanh B Nguyen; David Peretz; Fred E Cohen; Stephen J DeArmond; Stanley B Prusiner Continuum of prion protein structures enciphers a multitude of prion isolate-specified phenotypes. Proceedings of the National Academy of Sciences of the United States of America 2006;103(50):19105-10.

Jana Boy; Trygve B Leergaard; Thorsten Schmidt; Francis Odeh; Ulrike Bichelmeier; Silke Nuber; Carsten Holzmann; Andreas Wree; Stanley B Prusiner; Hermann Bujard; et al. Expression mapping of tetracycline-responsive prion protein promoter: digital atlasing for generating cell-specific disease models. NeuroImage 2006;33(2):449-62.

E Leclerc; H Serban; S B Prusiner; D R Burton; R A Williamson Copper induces conformational changes in the N-terminal part of cell-surface PrPC. Archives of virology 2006;151(11):2103-9.

Béatrice Luginbühl; Zoltan Kanyo; R Mark Jones; Robert J Fletterick; Stanley B Prusiner; Fred E Cohen; R Anthony Williamson; Dennis R Burton; Andreas Plückthun Directed evolution of an anti-prion protein scFv fragment to an affinity of 1 pM and its structural interpretation. Journal of molecular biology 2006;363(1):75-97.

Barnaby C H May; Juanita Witkop; John Sherrill; Marc O Anderson; Peter B Madrid; Julie A Zorn; Stanley B Prusiner; Fred E Cohen; R Kiplin Guy Structure-activity relationship study of 9-aminoacridine compounds in scrapie-infected neuroblastoma cells. Bioorganic & medicinal chemistry letters 2006;16(18):4913-6.

Gültekin Tamgüney; Kurt Giles; Essia Bouzamondo-Bernstein; Patrick J Bosque; Michael W Miller; Jiri Safar; Stephen J DeArmond; Stanley B Prusiner Transmission of elk and deer prions to transgenic mice. Journal of virology 2006;80(18):9104-14.

Jiri G Safar; Holger Wille; Michael D Geschwind; Camille Deering; Diane Latawiec; Ana Serban; David J King; Giuseppe Legname; Karl H Weisgraber; Robert W Mahley; et al. Human prions and plasma lipoproteins. Proceedings of the National Academy of Sciences of the United States of America 2006;103(30):11312-7.

Yong Huang; Hideaki Okochi; Barnaby C H May; Giuseppe Legname; Stanley B Prusiner; Leslie Z Benet; B Joseph Guglielmo; Emil T Lin Quinacrine is mainly metabolized to mono-desethyl quinacrine by CYP3A4/5 and its brain accumulation is limited by P-glycoprotein. Drug metabolism and disposition: the biological fate of chemicals 2006;34(7):1136-44.

Ranjit K Giri; Rebecca Young; Rose Pitstick; Stephen J DeArmond; Stanley B Prusiner; George A Carlson Prion infection of mouse neurospheres. Proceedings of the National Academy of Sciences of the United States of America 2006;103(10):3875-80.

Kwang Hun Lim; Tuan N Nguyen; Steven M Damo; Tanya Mazur; Haydn L Ball; Stanley B Prusiner; Alexander Pines; David E Wemmer Solid-state NMR structural studies of the fibril form of a mutant mouse prion peptide PrP89-143(P101L). Solid state nuclear magnetic resonance 2006;29(1-3):183-90.

Stanley B Prusiner; Maclyn McCarty Discovering DNA encodes heredity and prions are infectious proteins. Annual review of genetics 2006;40():25-45.

David Peretz; Surachai Supattapone; Kurt Giles; Julie Vergara; Yevgeniy Freyman; Pierre Lessard; Jiri G Safar; David V Glidden; Charles McCulloch; Hoang-Oanh B Nguyen; et al. Inactivation of prions by acidic sodium dodecyl sulfate. Journal of virology 2006;80(1):322-31.

Jamil Kanaani; Stanley B Prusiner; Julia Diacovo; Steinunn Baekkeskov; Giuseppe Legname Recombinant prion protein induces rapid polarization and development of synapses in embryonic rat hippocampal neurons in vitro. Journal of neurochemistry 2005;95(5):1373-86.

In Su Lee; Jeffrey R Long; Stanley B Prusiner; Jiri G Safar Selective precipitation of prions by polyoxometalate complexes. Journal of the American Chemical Society 2005;127(40):13802-3.

Jiri G Safar; Stephen J DeArmond; Katarzyna Kociuba; Camille Deering; Svetlana Didorenko; Essia Bouzamondo-Bernstein; Stanley B Prusiner; Patrick Tremblay Prion clearance in bigenic mice. The Journal of general virology 2005;86(Pt 10):2913-23.

Jiri G Safar; Klaus Kellings; Ana Serban; Darlene Groth; James E Cleaver; Stanley B Prusiner; Detlev Riesner Search for a prion-specific nucleic acid. Journal of virology 2005;79(16):10796-806.

Karl-Werner Leffers; Holger Wille; Jan Stöhr; Erika Junger; Stanley B Prusiner; Detlev Riesner Assembly of natural and recombinant prion protein into fibrils. Biological chemistry 2005;386(6):569-80.

Michael R Scott; David Peretz; Hoang-Oanh B Nguyen; Stephen J Dearmond; Stanley B Prusiner Transmission barriers for bovine, ovine, and human prions in transgenic mice. Journal of virology 2005;79(9):5259-71.

Jiri G Safar; Michael D Geschwind; Camille Deering; Svetlana Didorenko; Mamta Sattavat; Henry Sanchez; Ana Serban; Martin Vey; Henry Baron; Kurt Giles; et al. Diagnosis of human prion disease. Proceedings of the National Academy of Sciences of the United States of America 2005;102(9):3501-6.

Giuseppe Legname; Hoang-Oanh B Nguyen; Ilia V Baskakov; Fred E Cohen; Stephen J Dearmond; Stanley B Prusiner Strain-specified characteristics of mouse synthetic prions. Proceedings of the National Academy of Sciences of the United States of America 2005;102(6):2168-73.

Nako Ishikura; Jared L Clever; Essia Bouzamondo-Bernstein; Erik Samayoa; Stanley B Prusiner; Eric J Huang; Stephen J DeArmond Notch-1 activation and dendritic atrophy in prion disease. Proceedings of the National Academy of Sciences of the United States of America 2005;102(3):886-91.

Jesús R Requena; Mariana N Dimitrova; Giuseppe Legname; Susana Teijeira; Stanley B Prusiner; Rodney L Levine Oxidation of methionine residues in the prion protein by hydrogen peroxide. Archives of biochemistry and biophysics 2004;432(2):188-95.

Lotus Yung; Yong Huang; Pierre Lessard; Giuseppe Legname; Emil T Lin; Michael Baldwin; Stanley B Prusiner; Chongsuk Ryou; B Joseph Guglielmo Pharmacokinetics of quinacrine in the treatment of prion disease. BMC infectious diseases 2004;4():53.

Ana Serban; Giuseppe Legname; Kirk Hansen; Nadia Kovaleva; Stanley B Prusiner Immunoglobulins in urine of hamsters with scrapie. The Journal of biological chemistry 2004;279(47):48817-20.

Essia Bouzamondo-Bernstein; Stephanie D Hopkins; Patricia Spilman; Jane Uyehara-Lock; Camille Deering; Jiri Safar; Stanley B Prusiner; Henry J Ralston; Stephen J DeArmond The neurodegeneration sequence in prion diseases: evidence from functional, morphological and ultrastructural studies of the GABAergic system. Journal of neuropathology and experimental neurology 2004;63(8):882-99.

Giuseppe Legname; Ilia V Baskakov; Hoang-Oanh B Nguyen; Detlev Riesner; Fred E Cohen; Stephen J DeArmond; Stanley B Prusiner Synthetic mammalian prions. Science (New York, N.Y.) 2004;305(5684):673-6.

Stanley B Prusiner Detecting mad cow disease. Scientific American 2004;291(1):86-93.

Cédric Govaerts; Holger Wille; Stanley B Prusiner; Fred E Cohen Evidence for assembly of prions with left-handed beta-helices into trimers. Proceedings of the National Academy of Sciences of the United States of America 2004;101(22):8342-7.

Gerold Schmitt-Ulms; Kirk Hansen; Jialing Liu; Cynthia Cowdrey; Jian Yang; Stephen J DeArmond; Fred E Cohen; Stanley B Prusiner; Michael A Baldwin Time-controlled transcardiac perfusion cross-linking for the study of protein interactions in complex tissues. Nature biotechnology 2004;22(6):724-31.

Barnaby C H May; Cedric Govaerts; Stanley B Prusiner; Fred E Cohen Prions: so many fibers, so little infectivity. Trends in biochemical sciences 2004;29(4):162-5.

Ilia V Baskakov; Giuseppe Legname; Zygmunt Gryczynski; Stanley B Prusiner The peculiar nature of unfolding of the human prion protein. Protein science : a publication of the Protein Society 2004;13(3):586-95.

Jill S Goldman; Bruce L Miller; Jiri Safar; Sunita de Tourreil; Jennifer L Martindale; Stanley B Prusiner; Michael D Geschwind When sporadic disease is not sporadic: the potential for genetic etiology. Archives of neurology 2004;61(2):213-6.

Patrick Tremblay; Haydn L Ball; Kiyotoshi Kaneko; Darlene Groth; Ramanujan S Hegde; Fred E Cohen; Stephen J DeArmond; Stanley B Prusiner; Jiri G Safar Mutant PrPSc conformers induced by a synthetic peptide and several prion strains. Journal of virology 2004;78(4):2088-99.

Stanley B Prusiner Early evidence that a protease-resistant protein is an active component of the infectious prion. Cell 2004;116(2 Suppl):S109, 1 p following S113.

Peter J Peters; Alexander Mironov; David Peretz; Elly van Donselaar; Estelle Leclerc; Susanne Erpel; Stephen J DeArmond; Dennis R Burton; R Anthony Williamson; Martin Vey; et al. Trafficking of prion proteins through a caveolae-mediated endosomal pathway. The Journal of cell biology 2003;162(4):703-17.

Alexander Mironov; Diane Latawiec; Holger Wille; Essia Bouzamondo-Bernstein; Giuseppe Legname; R Anthony Williamson; Dennis Burton; Stephen J DeArmond; Stanley B Prusiner; Peter J Peters Cytosolic prion protein in neurons. The Journal of neuroscience : the official journal of the Society for Neuroscience 2003;23(18):7183-93.

Colin S Burns; Eliah Aronoff-Spencer; Giuseppe Legname; Stanley B Prusiner; William E Antholine; Gary J Gerfen; Jack Peisach; Glenn L Millhauser Copper coordination in the full-length, recombinant prion protein. Biochemistry 2003;42(22):6794-803.

Chongsuk Ryou; Giuseppe Legname; David Peretz; John C Craig; Michael A Baldwin; Stanley B Prusiner Differential inhibition of prion propagation by enantiomers of quinacrine. Laboratory investigation; a journal of technical methods and pathology 2003;83(6):837-43.

Chongsuk Ryou; Stanley B Prusiner; Giuseppe Legname Cooperative binding of dominant-negative prion protein to kringle domains. Journal of molecular biology 2003;329(2):323-33.

Carsten Korth; Kiyotoshi Kaneko; Darlene Groth; Norbert Heye; Glenn Telling; James Mastrianni; Piero Parchi; Pierluigi Gambetti; Robert Will; James Ironside; et al. Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene. Proceedings of the National Academy of Sciences of the United States of America 2003;100(8):4784-9.

Barnaby C H May; Aaron T Fafarman; Septima B Hong; Michael Rogers; Leslie W Deady; Stanley B Prusiner; Fred E Cohen Potent inhibition of scrapie prion replication in cultured cells by bis-acridines. Proceedings of the National Academy of Sciences of the United States of America 2003;100(6):3416-21.

Kefeng Qin; Janaky Coomaraswamy; Peter Mastrangelo; Ying Yang; Stan Lugowski; Chris Petromilli; Stanley B Prusiner; Paul E Fraser; Jonathan M Goldberg; Avijit Chakrabartty; et al. The PrP-like protein Doppel binds copper. The Journal of biological chemistry 2003;278(11):8888-96.

Stephen J DeArmond; Stanley B Prusiner Perspectives on prion biology, prion disease pathogenesis, and pharmacologic approaches to treatment. Clinics in laboratory medicine 2003;23(1):1-41.

Estelle Leclerc; David Peretz; Haydn Ball; Laura Solforosi; Giuseppe Legname; Jiri Safar; Ana Serban; Stanley B Prusiner; Dennis R Burton; R Anthony Williamson Conformation of PrP(C) on the cell surface as probed by antibodies. Journal of molecular biology 2003;326(2):475-83.

Giuseppe Legname; Peter Nelken; Zhengyu Guan; Zoltan F Kanyo; Stephen J DeArmond; Stanley B Prusiner Prion and doppel proteins bind to granule cells of the cerebellum. Proceedings of the National Academy of Sciences of the United States of America 2002;99(25):16285-90.

Stanley B Prusiner Historical essay. Discovering the cause of AIDS. Science (New York, N.Y.) 2002;298(5599):1726.

Jiri G Safar; Michael Scott; Jeff Monaghan; Camille Deering; Svetlana Didorenko; Julie Vergara; Haydn Ball; Giuseppe Legname; Estelle Leclerc; Laura Solforosi; et al. Measuring prions causing bovine spongiform encephalopathy or chronic wasting disease by immunoassays and transgenic mice. Nature biotechnology 2002;20(11):1147-50.

Kazuo Kuwata; Hua Li; Hiroaki Yamada; Giuseppe Legname; Stanley B Prusiner; Kazuyuki Akasaka; Thomas L James Locally disordered conformer of the hamster prion protein: a crucial intermediate to PrPSc? Biochemistry 2002;41(41):12277-83.

Véronique Perrier; Kiyotoshi Kaneko; Jiri Safar; Julie Vergara; Patrick Tremblay; Stephen J DeArmond; Fred E Cohen; Stanley B Prusiner; Andrew C Wallace Dominant-negative inhibition of prion replication in transgenic mice. Proceedings of the National Academy of Sciences of the United States of America 2002;99(20):13079-84.

Ilia V Baskakov; Giuseppe Legname; Michael A Baldwin; Stanley B Prusiner; Fred E Cohen Pathway complexity of prion protein assembly into amyloid. The Journal of biological chemistry 2002;277(24):21140-8.

David Peretz; R Anthony Williamson; Giuseppe Legname; Yoichi Matsunaga; Julie Vergara; Dennis R Burton; Stephen J DeArmond; Stanley B Prusiner; Michael R Scott A change in the conformation of prions accompanies the emergence of a new prion strain. Neuron 2002;34(6):921-32.

Patrick J Bosque; Chongsuk Ryou; Glenn Telling; David Peretz; Giuseppe Legname; Stephen J DeArmond; Stanley B Prusiner Prions in skeletal muscle. Proceedings of the National Academy of Sciences of the United States of America 2002;99(6):3812-7.

Holger Wille; Melissa D Michelitsch; Vincent Guenebaut; Surachai Supattapone; Ana Serban; Fred E Cohen; David A Agard; Stanley B Prusiner Structural studies of the scrapie prion protein by electron crystallography. Proceedings of the National Academy of Sciences of the United States of America 2002;99(6):3563-8.

Eric M Nicholson; Huaping Mo; Stanley B Prusiner; Fred E Cohen; Susan Marqusee Differences between the prion protein and its homolog Doppel: a partially structured state with implications for scrapie formation. Journal of molecular biology 2002;316(3):807-15.

J A Mastrianni; S Capellari; G C Telling; D Han; P Bosque; S B Prusiner; S J DeArmond Inherited prion disease caused by the V210I mutation: transmission to transgenic mice. Neurology 2001;57(12):2198-205.

R C Moore; P Mastrangelo; E Bouzamondo; C Heinrich; G Legname; S B Prusiner; L Hood; D Westaway; S J DeArmond; P Tremblay Doppel-induced cerebellar degeneration in transgenic mice. Proceedings of the National Academy of Sciences of the United States of America 2001;98(26):15288-93.

G Schmitt-Ulms; G Legname; M A Baldwin; H L Ball; N Bradon; P J Bosque; K L Crossin; G M Edelman; S J DeArmond; F E Cohen; et al. Binding of neural cell adhesion molecules (N-CAMs) to the cellular prion protein. Journal of molecular biology 2001;314(5):1209-25.

R C Moore; F Xiang; J Monaghan; D Han; Z Zhang; L Edström; M Anvret; S B Prusiner Huntington disease phenocopy is a familial prion disease. American journal of human genetics 2001;69(6):1385-8.

H L Ball; D S King; F E Cohen; S B Prusiner; M A Baldwin Engineering the prion protein using chemical synthesis. The journal of peptide research : official journal of the American Peptide Society 2001;58(5):357-74.

M Schlumpberger; S B Prusiner; I Herskowitz Induction of distinct [URE3] yeast prion strains. Molecular and cellular biology 2001;21(20):7035-46.

D D Laws; H M Bitter; K Liu; H L Ball; K Kaneko; H Wille; F E Cohen; S B Prusiner; A Pines; D E Wemmer Solid-state NMR studies of the secondary structure of a mutant prion protein fragment of 55 residues that induces neurodegeneration. Proceedings of the National Academy of Sciences of the United States of America 2001;98(20):11686-90.

D Peretz; R A Williamson; K Kaneko; J Vergara; E Leclerc; G Schmitt-Ulms; I R Mehlhorn; G Legname; M R Wormald; P M Rudd; et al. Antibodies inhibit prion propagation and clear cell cultures of prion infectivity. Nature 2001;412(6848):739-43.

C Korth; B C May; F E Cohen; S B Prusiner Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease. Proceedings of the National Academy of Sciences of the United States of America 2001;98(17):9836-41.

M Diez; S J DeArmond; D Groth; S B Prusiner; T Hökfelt Decreased MK-801 binding in discrete hippocampal regions of prion-infected mice. Neurobiology of disease 2001;8(4):692-9.

Y Matsunaga; D Peretz; A Williamson; D Burton; I Mehlhorn; D Groth; F E Cohen; S B Prusiner; M A Baldwin Cryptic epitopes in N-terminally truncated prion protein are exposed in the full-length molecule: dependence of conformation on pH. Proteins 2001;44(2):110-8.

J R Requena; D Groth; G Legname; E R Stadtman; S B Prusiner; R L Levine Copper-catalyzed oxidation of the recombinant SHa(29-231) prion protein. Proceedings of the National Academy of Sciences of the United States of America 2001;98(13):7170-5.

I V Baskakov; G Legname; S B Prusiner; F E Cohen Folding of prion protein to its native alpha-helical conformation is under kinetic control. The Journal of biological chemistry 2001;276(23):19687-90.

S B Prusiner Shattuck lecture–neurodegenerative diseases and prions. The New England journal of medicine 2001;344(20):1516-26.

P M Rudd; M R Wormald; D R Wing; S B Prusiner; R A Dwek Prion glycoprotein: structure, dynamics, and roles for the sugars. Biochemistry 2001;40(13):3759-66.

E Leclerc; D Peretz; H Ball; H Sakurai; G Legname; A Serban; S B Prusiner; D R Burton; R A Williamson Immobilized prion protein undergoes spontaneous rearrangement to a conformation having features in common with the infectious form. The EMBO journal 2001;20(7):1547-54.

K Jansen; O Schäfer; E Birkmann; K Post; H Serban; S B Prusiner; D Riesner Structural intermediates in the putative pathway from the cellular prion protein to the pathogenic form. Biological chemistry 2001;382(4):683-91.

D Peretz; M R Scott; D Groth; R A Williamson; D R Burton; F E Cohen; S B Prusiner Strain-specified relative conformational stability of the scrapie prion protein. Protein science : a publication of the Protein Society 2001;10(4):854-63.

P M Harrison; H S Chan; S B Prusiner; F E Cohen Conformational propagation with prion-like characteristics in a simple model of protein folding. Protein science : a publication of the Protein Society 2001;10(4):819-35.

S Supattapone; E Bouzamondo; H L Ball; H Wille; H O Nguyen; F E Cohen; S J DeArmond; S B Prusiner; M Scott A protease-resistant 61-residue prion peptide causes neurodegeneration in transgenic mice. Molecular and cellular biology 2001;21(7):2608-16.

S Supattapone; H Wille; L Uyechi; J Safar; P Tremblay; F C Szoka; F E Cohen; S B Prusiner; M R Scott Branched polyamines cure prion-infected neuroblastoma cells. Journal of virology 2001;75(7):3453-61.

J H Viles; D Donne; G Kroon; S B Prusiner; F E Cohen; H J Dyson; P E Wright Local structural plasticity of the prion protein. Analysis of NMR relaxation dynamics. Biochemistry 2001;40(9):2743-53.

H Mo; R C Moore; F E Cohen; D Westaway; S B Prusiner; P E Wright; H J Dyson Two different neurodegenerative diseases caused by proteins with similar structures. Proceedings of the National Academy of Sciences of the United States of America 2001;98(5):2352-7.

S Supattapone; T Muramoto; G Legname; I Mehlhorn; F E Cohen; S J DeArmond; S B Prusiner; M R Scott Identification of two prion protein regions that modify scrapie incubation time. Journal of virology 2001;75(3):1408-13.

S Supattapone; H O Nguyen; T Muramoto; F E Cohen; S J DeArmond; S B Prusiner; M Scott Affinity-tagged miniprion derivatives spontaneously adopt protease-resistant conformations. Journal of virology 2000;74(24):11928-34.

E Aronoff-Spencer; C S Burns; N I Avdievich; G J Gerfen; J Peisach; W E Antholine; H L Ball; F E Cohen; S B Prusiner; G L Millhauser Identification of the Cu2+ binding sites in the N-terminal domain of the prion protein by EPR and CD spectroscopy. Biochemistry 2000;39(45):13760-71.

D A Stephenson; K Chiotti; C Ebeling; D Groth; S J DeArmond; S B Prusiner; G A Carlson Quantitative trait loci affecting prion incubation time in mice. Genomics 2000;69(1):47-53.

C Korth; K Kaneko; S B Prusiner Expression of unglycosylated mutated prion protein facilitates PrP(Sc) formation in neuroblastoma cells infected with different prion strains. The Journal of general virology 2000;81(Pt 10):2555-63.

G L Silverman; K Qin; R C Moore; Y Yang; P Mastrangelo; P Tremblay; S B Prusiner; F E Cohen; D Westaway Doppel is an N-glycosylated, glycosylphosphatidylinositol-anchored protein. Expression in testis and ectopic production in the brains of Prnp(0/0) mice predisposed to Purkinje cell loss. The Journal of biological chemistry 2000;275(35):26834-41.

E Bouzamondo; A M Milroy; H J Ralston; S B Prusiner; S J DeArmond Selective neuronal vulnerability during experimental scrapie infection: insights from an ultrastructural investigation. Brain research 2000;874(2):210-5.

H Inouye; J Bond; M A Baldwin; H L Ball; S B Prusiner; D A Kirschner Structural changes in a hydrophobic domain of the prion protein induced by hydration and by ala–>Val and pro–>Leu substitutions. Journal of molecular biology 2000;300(5):1283-96.

H Wille; S B Prusiner; F E Cohen Scrapie infectivity is independent of amyloid staining properties of the N-terminally truncated prion protein. Journal of structural biology 2000;130(2-3):323-38.

V Perrier; A C Wallace; K Kaneko; J Safar; S B Prusiner; F E Cohen Mimicking dominant negative inhibition of prion replication through structure-based drug design. Proceedings of the National Academy of Sciences of the United States of America 2000;97(11):6073-8.

P J Bosque; S B Prusiner Cultured cell sublines highly susceptible to prion infection. Journal of virology 2000;74(9):4377-86.

L Zulianello; K Kaneko; M Scott; S Erpel; D Han; F E Cohen; S B Prusiner Dominant-negative inhibition of prion formation diminished by deletion mutagenesis of the prion protein. Journal of virology 2000;74(9):4351-60.

I V Baskakov; C Aagaard; I Mehlhorn; H Wille; D Groth; M A Baldwin; S B Prusiner; F E Cohen Self-assembly of recombinant prion protein of 106 residues. Biochemistry 2000;39(10):2792-804.

M Schlumpberger; H Wille; M A Baldwin; D A Butler; I Herskowitz; S B Prusiner The prion domain of yeast Ure2p induces autocatalytic formation of amyloid fibers by a recombinant fusion protein. Protein science : a publication of the Protein Society 2000;9(3):440-51.

R M Whittal; H L Ball; F E Cohen; A L Burlingame; S B Prusiner; M A Baldwin Copper binding to octarepeat peptides of the prion protein monitored by mass spectrometry. Protein science : a publication of the Protein Society 2000;9(2):332-43.

K Kaneko; H L Ball; H Wille; H Zhang; D Groth; M Torchia; P Tremblay; J Safar; S B Prusiner; S J DeArmond; et al. A synthetic peptide initiates Gerstmann-Sträussler-Scheinker (GSS) disease in transgenic mice. Journal of molecular biology 2000;295(4):997-1007.

J Safar; F E Cohen; S B Prusiner Quantitative traits of prion strains are enciphered in the conformation of the prion protein. Archives of virology. Supplementum 2000;(16):227-35.

M R Scott; S Supattapone; H O Nguyen; S J DeArmond; S B Prusiner Transgenic models of prion disease. Archives of virology. Supplementum 2000;(16):113-24.

M R Scott; R Will; J Ironside; H O Nguyen; P Tremblay; S J DeArmond; S B Prusiner Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proceedings of the National Academy of Sciences of the United States of America 1999;96(26):15137-42.

R S Hegde; P Tremblay; D Groth; S J DeArmond; S B Prusiner; V R Lingappa Transmissible and genetic prion diseases share a common pathway of neurodegeneration. Nature 1999;402(6763):822-6.

S Supattapone; H O Nguyen; F E Cohen; S B Prusiner; M R Scott Elimination of prions by branched polyamines and implications for therapeutics. Proceedings of the National Academy of Sciences of the United States of America 1999;96(25):14529-34.

J Tatzelt; D F Groth; M Torchia; S B Prusiner; S J DeArmond Kinetics of prion protein accumulation in the CNS of mice with experimental scrapie. Journal of neuropathology and experimental neurology 1999;58(12):1244-9.

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. Glycosylation differences between the normal and pathogenic prion protein isoforms. Proceedings of the National Academy of Sciences of the United States of America 1999;96(23):13044-9.

Z F Kanyo; K M Pan; R A Williamson; D R Burton; S B Prusiner; R J Fletterick; F E Cohen Antibody binding defines a structure for an epitope that participates in the PrPC–>PrPSc conformational change. Journal of molecular biology 1999;293(4):855-63.

R C Moore; I Y Lee; G L Silverman; P M Harrison; R Strome; C Heinrich; A Karunaratne; S H Pasternak; M A Chishti; Y Liang; et al. Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel. Journal of molecular biology 1999;292(4):797-817.

N Nishida; P Tremblay; T Sugimoto; K Shigematsu; S Shirabe; C Petromilli; S P Erpel; R Nakaoke; R Atarashi; T Houtani; et al. A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination. Laboratory investigation; a journal of technical methods and pathology 1999;79(6):689-97.

J A Mastrianni; R Nixon; R Layzer; G C Telling; D Han; S J DeArmond; S B Prusiner Prion protein conformation in a patient with sporadic fatal insomnia. The New England journal of medicine 1999;340(21):1630-8.

H Liu; S Farr-Jones; N B Ulyanov; M Llinas; S Marqusee; D Groth; F E Cohen; S B Prusiner; T L James Solution structure of Syrian hamster prion protein rPrP(90-231). Biochemistry 1999;38(17):5362-77.

S Supattapone; P Bosque; T Muramoto; H Wille; C Aagaard; D Peretz; H O Nguyen; C Heinrich; M Torchia; J Safar; et al. Prion protein of 106 residues creates an artifical transmission barrier for prion replication in transgenic mice. Cell 1999;96(6):869-78.

J H Viles; F E Cohen; S B Prusiner; D B Goodin; P E Wright; H J Dyson Copper binding to the prion protein: structural implications of four identical cooperative binding sites. Proceedings of the National Academy of Sciences of the United States of America 1999;96(5):2042-7.

P M Harrison; H S Chan; S B Prusiner; F E Cohen Thermodynamics of model prions and its implications for the problem of prion protein folding. Journal of molecular biology 1999;286(2):593-606.

H Wille; S B Prusiner Ultrastructural studies on scrapie prion protein crystals obtained from reverse micellar solutions. Biophysical journal 1999;76(2):1048-62.

S B Prusiner Prions. Proceedings of the National Academy of Sciences of the United States of America 1998;95(23):13363-83.

K Post; M Pitschke; O Schäfer; H Wille; T R Appel; D Kirsch; I Mehlhorn; H Serban; S B Prusiner; D Riesner Rapid acquisition of beta-sheet structure in the prion protein prior to multimer formation. Biological chemistry 1998;379(11):1307-17.

R A Williamson; D Peretz; C Pinilla; H Ball; R B Bastidas; R Rozenshteyn; R A Houghten; S B Prusiner; D R Burton Mapping the prion protein using recombinant antibodies. Journal of virology 1998;72(11):9413-8.

P Tremblay; Z Meiner; M Galou; C Heinrich; C Petromilli; T Lisse; J Cayetano; M Torchia; W Mobley; H Bujard; et al. Doxycycline control of prion protein transgene expression modulates prion disease in mice. Proceedings of the National Academy of Sciences of the United States of America 1998;95(21):12580-5.

I Y Lee; D Westaway; A F Smit; K Wang; J Seto; L Chen; C Acharya; M Ankener; D Baskin; C Cooper; et al. Complete genomic sequence and analysis of the prion protein gene region from three mammalian species. Genome research 1998;8(10):1022-37.

J Safar; H Wille; V Itri; D Groth; H Serban; M Torchia; F E Cohen; S B Prusiner Eight prion strains have PrP(Sc) molecules with different conformations. Nature medicine 1998;4(10):1157-65.

M A Baldwin; T L James; F E Cohen; S B Prusiner The three-dimensional structure of prion protein: implications for prion disease. Biochemical Society transactions 1998;26(3):481-6.

S B Prusiner The prion diseases. Brain pathology (Zurich, Switzerland) 1998;8(3):499-513.

J Stöckel; J Safar; A C Wallace; F E Cohen; S B Prusiner Prion protein selectively binds copper(II) ions. Biochemistry 1998;37(20):7185-93.

T Hökfelt; C Broberger; X Zhang; M Diez; J Kopp; Z Xu; M Landry; L Bao; M Schalling; J Koistinaho; et al. Neuropeptide Y: some viewpoints on a multifaceted peptide in the normal and diseased nervous system. Brain research. Brain research reviews 1998;26(2-3):154-66.

S B Prusiner; M R Scott; S J DeArmond; F E Cohen Prion protein biology. Cell 1998;93(3):337-48.

R S Hegde; J A Mastrianni; M R Scott; K A DeFea; P Tremblay; M Torchia; S J DeArmond; S B Prusiner; V R Lingappa A transmembrane form of the prion protein in neurodegenerative disease. Science (New York, N.Y.) 1998;279(5352):827-34.

J Safar; S B Prusiner Molecular studies of prion diseases. Progress in brain research 1998;117():421-34.

F E Cohen; S B Prusiner Pathologic conformations of prion proteins. Annual review of biochemistry 1998;67():793-819.

S J DeArmond; W C Mobley; D L DeMott; R A Barry; J H Beckstead; S B Prusiner Changes in the localization of brain prion proteins during scrapie infection. Neurology 1998;50(1):1271-80; discussion 2.

M R Scott; J Safar; G Telling; O Nguyen; D Groth; M Torchia; R Koehler; P Tremblay; D Walther; F E Cohen; et al. Identification of a prion protein epitope modulating transmission of bovine spongiform encephalopathy prions to transgenic mice. Proceedings of the National Academy of Sciences of the United States of America 1997;94(26):14279-84.

D G Donne; J H Viles; D Groth; I Mehlhorn; T L James; F E Cohen; S B Prusiner; P E Wright; H J Dyson Structure of the recombinant full-length hamster prion protein PrP(29-231): the N terminus is highly flexible. Proceedings of the National Academy of Sciences of the United States of America 1997;94(25):13452-7.

T C Blochberger; C Cooper; D Peretz; J Tatzelt; O H Griffith; M A Baldwin; S B Prusiner Prion protein expression in Chinese hamster ovary cells using a glutamine synthetase selection and amplification system. Protein engineering 1997;10(12):1465-73.

S J DeArmond; H Sánchez; F Yehiely; Y Qiu; A Ninchak-Casey; V Daggett; A P Camerino; J Cayetano; M Rogers; D Groth; et al. Selective neuronal targeting in prion disease. Neuron 1997;19(6):1337-48.

M R Scott; D Groth; J Tatzelt; M Torchia; P Tremblay; S J DeArmond; S B Prusiner Propagation of prion strains through specific conformers of the prion protein. Journal of virology 1997;71(12):9032-44.

M Diez; J Koistinaho; S J Dearmond; D Groth; S B Prusiner; T Hökfelt Marked decrease of neuropeptide Y Y2 receptor binding sites in the hippocampus in murine prion disease. Proceedings of the National Academy of Sciences of the United States of America 1997;94(24):13267-72.

H M Schätzl; L Laszlo; D M Holtzman; J Tatzelt; S J DeArmond; R I Weiner; W C Mobley; S B Prusiner A hypothalamic neuronal cell line persistently infected with scrapie prions exhibits apoptosis. Journal of virology 1997;71(11):8821-31.

D Peretz; R A Williamson; Y Matsunaga; H Serban; C Pinilla; R B Bastidas; R Rozenshteyn; T L James; R A Houghten; F E Cohen; et al. A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform. Journal of molecular biology 1997;273(3):614-22.

S B Prusiner Prion diseases and the BSE crisis. Science (New York, N.Y.) 1997;278(5336):245-51.

T L James; H Liu; N B Ulyanov; S Farr-Jones; H Zhang; D G Donne; K Kaneko; D Groth; I Mehlhorn; S B Prusiner; et al. Solution structure of a 142-residue recombinant prion protein corresponding to the infectious fragment of the scrapie isoform. Proceedings of the National Academy of Sciences of the United States of America 1997;94(19):10086-91.

K Kaneko; L Zulianello; M Scott; C M Cooper; A C Wallace; T L James; F E Cohen; S B Prusiner Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation. Proceedings of the National Academy of Sciences of the United States of America 1997;94(19):10069-74.

K Kaneko; H Wille; I Mehlhorn; H Zhang; H Ball; F E Cohen; M A Baldwin; S B Prusiner Molecular properties of complexes formed between the prion protein and synthetic peptides. Journal of molecular biology 1997;270(4):574-86.

Z Meiner; R Gabizon; S B Prusiner Familial Creutzfeldt-Jakob disease. Codon 200 prion disease in Libyan Jews. Medicine 1997;76(4):227-37.

T Muramoto; S J DeArmond; M Scott; G C Telling; F E Cohen; S B Prusiner Heritable disorder resembling neuronal storage disease in mice expressing prion protein with deletion of an alpha-helix. Nature medicine 1997;3(7):750-5.

K M Lundberg; C J Stenland; F E Cohen; S B Prusiner; G L Millhauser Kinetics and mechanism of amyloid formation by the prion protein H1 peptide as determined by time-dependent ESR. Chemistry & biology 1997;4(5):345-55.

G C Telling; P Tremblay; M Torchia; S J Dearmond; F E Cohen; S B Prusiner N-terminally tagged prion protein supports prion propagation in transgenic mice. Protein science : a publication of the Protein Society 1997;6(4):825-33.

H Zhang; J Stockel; I Mehlhorn; D Groth; M A Baldwin; S B Prusiner; T L James; F E Cohen Physical studies of conformational plasticity in a recombinant prion protein. Biochemistry 1997;36(12):3543-53.

K Kaneko; M Vey; M Scott; S Pilkuhn; F E Cohen; S B Prusiner COOH-terminal sequence of the cellular prion protein directs subcellular trafficking and controls conversion into the scrapie isoform. Proceedings of the National Academy of Sciences of the United States of America 1997;94(6):2333-8.

G A Carlson; S Banks; D Lund; C Reichert; D Groth; M Torchia; S J Dearmond; S B Prusiner Failure to transmit disease from gray tremor mutant mice. Journal of virology 1997;71(3):2342-5.

P M Harrison; P Bamborough; V Daggett; S B Prusiner; F E Cohen The prion folding problem. Current opinion in structural biology 1997;7(1):53-9.

H M Schätzl; M Da Costa; L Taylor; F E Cohen; S B Prusiner Prion protein gene variation among primates. Journal of molecular biology 1997;265(2):257.

S B Prusiner; M R Scott Genetics of prions. Annual review of genetics 1997;31():139-75.

F Yehiely; P Bamborough; M Da Costa; B J Perry; G Thinakaran; F E Cohen; G A Carlson; S B Prusiner Identification of candidate proteins binding to prion protein. Neurobiology of disease 1997;3(4):339-55.

T Muramoto; M Scott; F E Cohen; S B Prusiner Recombinant scrapie-like prion protein of 106 amino acids is soluble. Proceedings of the National Academy of Sciences of the United States of America 1996;93(26):15457-62.

G C Telling; P Parchi; S J DeArmond; P Cortelli; P Montagna; R Gabizon; J Mastrianni; E Lugaresi; P Gambetti; S B Prusiner Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science (New York, N.Y.) 1996;274(5295):2079-82.

M Vey; S Pilkuhn; H Wille; R Nixon; S J DeArmond; E J Smart; R G Anderson; A Taraboulos; S B Prusiner Subcellular colocalization of the cellular and scrapie prion proteins in caveolae-like membranous domains. Proceedings of the National Academy of Sciences of the United States of America 1996;93(25):14945-9.

J Tatzelt; S B Prusiner; W J Welch Chemical chaperones interfere with the formation of scrapie prion protein. The EMBO journal 1996;15(23):6363-73.

S B Prusiner Molecular biology and pathogenesis of prion diseases. Trends in biochemical sciences 1996;21(12):482-7.

J A Mastrianni; C Iannicola; R M Myers; S DeArmond; S B Prusiner Mutation of the prion protein gene at codon 208 in familial Creutzfeldt-Jakob disease. Neurology 1996;47(5):1305-12.

S B Prusiner Prion biology and diseases–laughing cannibals, mad cows, and scientific heresy. Medicinal research reviews 1996;16(5):487-505.

K Wong; Y Qiu; W Hyun; R Nixon; J VanCleff; J Sanchez-Salazar; S B Prusiner; S J DeArmond Decreased receptor-mediated calcium response in prion-infected cells correlates with decreased membrane fluidity and IP3 release. Neurology 1996;47(3):741-50.

M Diez; J Koistinaho; S J DeArmond; A P Camerino; D Groth; J C Caytano; S B Prusiner; T Hökfelt Aberrant induction of neuropeptide Y mRNA in hippocampal CA3 pyramidal neurones in scrapie-infected mice. Neuroreport 1996;7(12):1887-92.

J Heller; A C Kolbert; R Larsen; M Ernst; T Bekker; M Baldwin; S B Prusiner; A Pines; D E Wemmer Solid-state NMR studies of the prion protein H1 fragment. Protein science : a publication of the Protein Society 1996;5(8):1655-61.

J Tatzelt; N Maeda; M Pekny; S L Yang; C Betsholtz; C Eliasson; J Cayetano; A P Camerino; S J DeArmond; S B Prusiner Scrapie in mice deficient in apolipoprotein E or glial fibrillary acidic protein. Neurology 1996;47(2):449-53.

G C Telling; T Haga; M Torchia; P Tremblay; S J DeArmond; S B Prusiner Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. Genes & development 1996;10(14):1736-50.

R A Williamson; D Peretz; N Smorodinsky; R Bastidas; H Serban; I Mehlhorn; S J DeArmond; S B Prusiner; D R Burton Circumventing tolerance to generate autologous monoclonal antibodies to the prion protein. Proceedings of the National Academy of Sciences of the United States of America 1996;93(14):7279-82.

H Wille; G F Zhang; M A Baldwin; F E Cohen; S B Prusiner Separation of scrapie prion infectivity from PrP amyloid polymers. Journal of molecular biology 1996;259(4):608-21.

I Mehlhorn; D Groth; J Stöckel; B Moffat; D Reilly; D Yansura; W S Willett; M Baldwin; R Fletterick; F E Cohen; et al. High-level expression and characterization of a purified 142-residue polypeptide of the prion protein. Biochemistry 1996;35(17):5528-37.

P M Lledo; P Tremblay; S J DeArmond; S B Prusiner; R A Nicoll Mice deficient for prion protein exhibit normal neuronal excitability and synaptic transmission in the hippocampus. Proceedings of the National Academy of Sciences of the United States of America 1996;93(6):2403-7.

D Riesner; K Kellings; K Post; H Wille; H Serban; D Groth; M A Baldwin; S B Prusiner Disruption of prion rods generates 10-nm spherical particles having high alpha-helical content and lacking scrapie infectivity. Journal of virology 1996;70(3):1714-22.

S J DeArmond; Y Qiu; K Wong; R Nixon; W Hyun; S B Prusiner; W C Mobley Abnormal plasma membrane properties and functions in prion-infected cell lines. Cold Spring Harbor symposia on quantitative biology 1996;61():531-40.

P Bamborough; H Wille; G C Telling; F Yehiely; S B Prusiner; F E Cohen Prion protein structure and scrapie replication: theoretical, spectroscopic, and genetic investigations. Cold Spring Harbor symposia on quantitative biology 1996;61():495-509.

S B Prusiner Molecular biology and genetics of prion diseases. Cold Spring Harbor symposia on quantitative biology 1996;61():473-93.

Z Huang; S B Prusiner; F E Cohen Scrapie prions: a three-dimensional model of an infectious fragment. Folding & design 1996;1(1):13-9.

H Wille; M A Baldwin; F E Cohen; S J DeArmond; S B Prusiner Prion protein amyloid: separation of scrapie infectivity from PrP polymers. Ciba Foundation symposium 1996;199():181-99; discussion 199-201.

S B Prusiner Transgenetics of prion diseases. Current topics in microbiology and immunology 1996;206():275-304.

M R Scott; G C Telling; S B Prusiner Transgenetics and gene targeting in studies of prion diseases. Current topics in microbiology and immunology 1996;207():95-123.

Z Huang; S B Prusiner; F E Cohen Structures of prion proteins and conformational models for prion diseases. Current topics in microbiology and immunology 1996;207():49-67.

S J DeArmond; S B Prusiner Transgenetics and neuropathology of prion diseases. Current topics in microbiology and immunology 1996;207():125-46.

S B Prusiner Human prion diseases and neurodegeneration. Current topics in microbiology and immunology 1996;207():1-17.

R Gabizon; G Telling; Z Meiner; M Halimi; I Kahana; S B Prusiner Insoluble wild-type and protease-resistant mutant prion protein in brains of patients with inherited prion disease. Nature medicine 1996;2(1):59-64.

Z Guan; M Söderberg; P Sindelar; S B Prusiner; K Kristensson; G Dallner Lipid composition in scrapie-infected mouse brain: prion infection increases the levels of dolichyl phosphate and ubiquinone. Journal of neurochemistry 1996;66(1):277-85.

K Kaneko; D Peretz; K M Pan; T C Blochberger; H Wille; R Gabizon; O H Griffith; F E Cohen; M A Baldwin; S B Prusiner Prion protein (PrP) synthetic peptides induce cellular PrP to acquire properties of the scrapie isoform. Proceedings of the National Academy of Sciences of the United States of America 1995;92(24):11160-4.

J A Mastrianni; M T Curtis; J C Oberholtzer; M M Da Costa; S DeArmond; S B Prusiner; J Y Garbern Prion disease (PrP-A117V) presenting with ataxia instead of dementia. Neurology 1995;45(11):2042-50.

G C Telling; M Scott; J Mastrianni; R Gabizon; M Torchia; F E Cohen; S J DeArmond; S B Prusiner Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell 1995;83(1):79-90.

U Wiese; M Wulfert; S B Prusiner; D Riesner Scanning for mutations in the human prion protein open reading frame by temporal temperature gradient gel electrophoresis. Electrophoresis 1995;16(10):1851-60.

J T Nguyen; H Inouye; M A Baldwin; R J Fletterick; F E Cohen; S B Prusiner; D A Kirschner X-ray diffraction of scrapie prion rods and PrP peptides. Journal of molecular biology 1995;252(4):412-22.

S Spudich; J A Mastrianni; M Wrensch; R Gabizon; Z Meiner; I Kahana; H Rosenmann; E Kahana; S B Prusiner Complete penetrance of Creutzfeldt-Jakob disease in Libyan Jews carrying the E200K mutation in the prion protein gene. Molecular medicine (Cambridge, Mass.) 1995;1(6):607-13.

M A Baldwin; F E Cohen; S B Prusiner Prion protein isoforms, a convergence of biological and structural investigations. The Journal of biological chemistry 1995;270(33):19197-200.

H Zhang; K Kaneko; J T Nguyen; T L Livshits; M A Baldwin; F E Cohen; T L James; S B Prusiner Conformational transitions in peptides containing two putative alpha-helices of the prion protein. Journal of molecular biology 1995;250(4):514-26.

S B Prusiner Molecular genetics and biophysics of prions. Uirusu 1995;45(1):5-42.

A J Raeber; T Muramoto; T B Kornberg; S B Prusiner Expression and targeting of Syrian hamster prion protein induced by heat shock in transgenic Drosophila melanogaster. Mechanisms of development 1995;51(2-3):317-27.

S L Kazmirski; D O Alonso; F E Cohen; S B Prusiner; V Daggett Theoretical studies of sequence effects on the conformational properties of a fragment of the prion protein: implications for scrapie formation. Chemistry & biology 1995;2(5):305-15.

J Nguyen; M A Baldwin; F E Cohen; S B Prusiner Prion protein peptides induce alpha-helix to beta-sheet conformational transitions. Biochemistry 1995;34(13):4186-92.

S J DeArmond; S B Prusiner Etiology and pathogenesis of prion diseases. The American journal of pathology 1995;146(4):785-811.

A Taraboulos; M Scott; A Semenov; D Avrahami; L Laszlo; S B Prusiner; D Avraham Cholesterol depletion and modification of COOH-terminal targeting sequence of the prion protein inhibit formation of the scrapie isoform. The Journal of cell biology 1995;129(1):121-32.

D Westaway; G A Carlson; S B Prusiner On safari with PrP: prion diseases of animals. Trends in microbiology 1995;3(4):141-7.

J Tatzelt; J Zuo; R Voellmy; M Scott; U Hartl; S B Prusiner; W J Welch Scrapie prions selectively modify the stress response in neuroblastoma cells. Proceedings of the National Academy of Sciences of the United States of America 1995;92(7):2944-8.

H M Schätzl; M Da Costa; L Taylor; F E Cohen; S B Prusiner Prion protein gene variation among primates. Journal of molecular biology 1995;245(4):362-74.

S B Prusiner The prion diseases. Scientific American 1995;272(1):48-51, 54-7.

S J DeArmond; S B Prusiner Prion protein transgenes and the neuropathology in prion diseases. Brain pathology (Zurich, Switzerland) 1995;5(1):77-89.

J G Jefferys; R M Empson; M A Whittington; S B Prusiner Scrapie infection of transgenic mice leads to network and intrinsic dysfunction of cortical and hippocampal neurones. Neurobiology of disease 1994;1(1-2):25-30.

G C Telling; M Scott; K K Hsiao; D Foster; S L Yang; M Torchia; K C Sidle; J Collinge; S J DeArmond; S B Prusiner Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein. Proceedings of the National Academy of Sciences of the United States of America 1994;91(21):9936-40.

K K Hsiao; D Groth; M Scott; S L Yang; H Serban; D Rapp; D Foster; M Torchia; S J Dearmond; S B Prusiner Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. Proceedings of the National Academy of Sciences of the United States of America 1994;91(19):9126-30.

S B Prusiner Neurodegeneration in humans caused by prions. The Western journal of medicine 1994;161(3):264-72.

Z Huang; J M Gabriel; M A Baldwin; R J Fletterick; S B Prusiner; F E Cohen Proposed three-dimensional structure for the cellular prion protein. Proceedings of the National Academy of Sciences of the United States of America 1994;91(15):7139-43.

D Westaway; C Cooper; S Turner; M Da Costa; G A Carlson; S B Prusiner Structure and polymorphism of the mouse prion protein gene. Proceedings of the National Academy of Sciences of the United States of America 1994;91(14):6418-22.

K A De Fea; D H Nakahara; M C Calayag; C S Yost; L F Mirels; S B Prusiner; V R Lingappa Determinants of carboxyl-terminal domain translocation during prion protein biogenesis. The Journal of biological chemistry 1994;269(24):16810-20.

G A Carlson; C Ebeling; S L Yang; G Telling; M Torchia; D Groth; D Westaway; S J DeArmond; S B Prusiner Prion isolate specified allotypic interactions between the cellular and scrapie prion proteins in congenic and transgenic mice. Proceedings of the National Academy of Sciences of the United States of America 1994;91(12):5690-4.

S B Prusiner Inherited prion diseases. Proceedings of the National Academy of Sciences of the United States of America 1994;91(11):4611-4.

F E Cohen; K M Pan; Z Huang; M Baldwin; R J Fletterick; S B Prusiner Structural clues to prion replication. Science (New York, N.Y.) 1994;264(5158):530-1.

D Westaway; V Zuliani; C M Cooper; M Da Costa; S Neuman; A L Jenny; L Detwiler; S B Prusiner Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie. Genes & development 1994;8(8):959-69.

S B Prusiner; K K Hsiao Human prion diseases. Annals of neurology 1994;35(4):385-95.

S B Prusiner Molecular biology and genetics of prion diseases. Philosophical transactions of the Royal Society of London. Series B, Biological sciences 1994;343(1306):447-63.

K Kellings; S B Prusiner; D Riesner Nucleic acids in prion preparations: unspecific background or essential component? Philosophical transactions of the Royal Society of London. Series B, Biological sciences 1994;343(1306):425-30.

S J DeArmond; S L Yang; J Cayetano-Canlas; D Groth; S B Prusiner The neuropathological phenotype in transgenic mice expressing different prion protein constructs. Philosophical transactions of the Royal Society of London. Series B, Biological sciences 1994;343(1306):415-23.

R Gabizon; H Rosenman; Z Meiner; I Kahana; E Kahana; Y Shugart; J Ott; S B Prusiner Mutation in codon 200 and polymorphism in codon 129 of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease. Philosophical transactions of the Royal Society of London. Series B, Biological sciences 1994;343(1306):385-90.

G A Carlson; S J DeArmond; M Torchia; D Westaway; S B Prusiner Genetics of prion diseases and prion diversity in mice. Philosophical transactions of the Royal Society of London. Series B, Biological sciences 1994;343(1306):363-9.

A Taraboulos; M Scott; A Semenov; D Avrahami; S B Prusiner Biosynthesis of the prion proteins in scrapie-infected cells in culture. Brazilian journal of medical and biological research = Revista brasileira de pesquisas médicas e biológicas / Sociedade Brasileira de Biofísica … [et al.] 1994;27(2):303-7.

H Gomi; T Ikeda; T Kunieda; S Itohara; S B Prusiner; K Yamanouchi Prion protein (PrP) is not involved in the pathogenesis of spongiform encephalopathy in zitter rats. Neuroscience letters 1994;166(2):171-4.

D Westaway; S J DeArmond; J Cayetano-Canlas; D Groth; D Foster; S L Yang; M Torchia; G A Carlson; S B Prusiner Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins. Cell 1994;76(1):117-29.

S B Prusiner; S J DeArmond Prion diseases and neurodegeneration. Annual review of neuroscience 1994;17():311-39.

J K Fink; M L Peacock; J T Warren; A D Roses; S B Prusiner Detecting prion protein gene mutations by denaturing gradient gel electrophoresis. Human mutation 1994;4(1):42-50.

S B Prusiner Biology and genetics of prion diseases. Annual review of microbiology 1994;48():655-86.

S B Prusiner A national strategy for development of effective methods for the prevention and treatment of Alzheimer’s disease and related neurodegenerative disorders. Neurobiology of aging 1994;15 Suppl 2():S29-32.

S B Prusiner; D Groth; A Serban; R Koehler; D Foster; M Torchia; D Burton; S L Yang; S J DeArmond Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies. Proceedings of the National Academy of Sciences of the United States of America 1993;90(22):10608-12.

K Kristensson; B Feuerstein; A Taraboulos; W C Hyun; S B Prusiner; S J DeArmond Scrapie prions alter receptor-mediated calcium responses in cultured cells. Neurology 1993;43(11):2335-41.

S B Prusiner Genetic and infectious prion diseases. Archives of neurology 1993;50(11):1129-53.

S J DeArmond; S B Prusiner The neurochemistry of prion diseases. Journal of neurochemistry 1993;61(5):1589-601.

S B Prusiner Transgenetics and cell biology of prion diseases: investigations of PrPSc synthesis and diversity. British medical bulletin 1993;49(4):873-912.

R Gabizon; H Rosenmann; Z Meiner; I Kahana; E Kahana; Y Shugart; J Ott; S B Prusiner Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease (CJD). American journal of human genetics 1993;53(4):828-35.

D R Borchelt; M Rogers; N Stahl; G Telling; S B Prusiner Release of the cellular prion protein from cultured cells after loss of its glycoinositol phospholipid anchor. Glycobiology 1993;3(4):319-29.

S J DeArmond; S L Yang; A Lee; R Bowler; A Taraboulos; D Groth; S B Prusiner Three scrapie prion isolates exhibit different accumulation patterns of the prion protein scrapie isoform. Proceedings of the National Academy of Sciences of the United States of America 1993;90(14):6449-53.

M Scott; D Groth; D Foster; M Torchia; S L Yang; S J DeArmond; S B Prusiner Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes. Cell 1993;73(5):979-88.

S B Prusiner Biology of prion diseases. Journal of acquired immune deficiency syndromes 1993;6(6):663-5.

M Rogers; F Yehiely; M Scott; S B Prusiner Conversion of truncated and elongated prion proteins into the scrapie isoform in cultured cells. Proceedings of the National Academy of Sciences of the United States of America 1993;90(8):3182-6.

S B Prusiner; D Groth; A Serban; N Stahl; R Gabizon Attempts to restore scrapie prion infectivity after exposure to protein denaturants. Proceedings of the National Academy of Sciences of the United States of America 1993;90(7):2793-7.

G A Carlson; C Ebeling; M Torchia; D Westaway; S B Prusiner Delimiting the location of the scrapie prion incubation time gene on chromosome 2 of the mouse. Genetics 1993;133(4):979-88.

N Stahl; M A Baldwin; D B Teplow; L Hood; B W Gibson; A L Burlingame; S B Prusiner Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing. Biochemistry 1993;32(8):1991-2002.

S B Prusiner; M Füzi; M Scott; D Serban; H Serban; A Taraboulos; J M Gabriel; G A Wells; J W Wilesmith; R Bradley Immunologic and molecular biologic studies of prion proteins in bovine spongiform encephalopathy. The Journal of infectious diseases 1993;167(3):602-13.

S B Prusiner Transgenetic investigations of prion diseases of humans and animals. Philosophical transactions of the Royal Society of London. Series B, Biological sciences 1993;339(1288):239-54.

M Gasset; M A Baldwin; R J Fletterick; S B Prusiner Perturbation of the secondary structure of the scrapie prion protein under conditions that alter infectivity. Proceedings of the National Academy of Sciences of the United States of America 1993;90(1):1-5.

S B Prusiner Prion encephalopathies of animals and humans. Developments in biological standardization 1993;80():31-44.

D Riesner; K Kellings; U Wiese; M Wulfert; C Mirenda; S B Prusiner Prions and nucleic acids: search for ”residual” nucleic acids and screening for mutations in the PrP-gene. Developments in biological standardization 1993;80():173-81.

K Kellings; N Meyer; C Mirenda; S B Prusiner; D Riesner Analysis of nucleic acids in purified scrapie prion preparations. Archives of virology. Supplementum 1993;7():215-25.

S B Prusiner Chemistry and biology of prions. Biochemistry 1992;31(49):12277-88.

M Gasset; M A Baldwin; D H Lloyd; J M Gabriel; D M Holtzman; F Cohen; R Fletterick; S B Prusiner Predicted alpha-helical regions of the prion protein when synthesized as peptides form amyloid. Proceedings of the National Academy of Sciences of the United States of America 1992;89(22):10940-4.

S B Prusiner Natural and experimental prion diseases of humans and animals. Current opinion in neurobiology 1992;2(5):638-47.

J M Gabriel; B Oesch; H Kretzschmar; M Scott; S B Prusiner Molecular cloning of a candidate chicken prion protein. Proceedings of the National Academy of Sciences of the United States of America 1992;89(19):9097-101.

H A Kretzschmar; M Neumann; G Riethmüller; S B Prusiner Molecular cloning of a mink prion protein gene. The Journal of general virology 1992;73 ( Pt 10)():2757-61.

K M Pan; N Stahl; S B Prusiner Purification and properties of the cellular prion protein from Syrian hamster brain. Protein science : a publication of the Protein Society 1992;1(10):1343-52.

G Giaccone; L Verga; O Bugiani; B Frangione; D Serban; S B Prusiner; M R Farlow; B Ghetti; F Tagliavini Prion protein preamyloid and amyloid deposits in Gerstmann-Sträussler-Scheinker disease, Indiana kindred. Proceedings of the National Academy of Sciences of the United States of America 1992;89(19):9349-53.

A J Raeber; D R Borchelt; M Scott; S B Prusiner Attempts to convert the cellular prion protein into the scrapie isoform in cell-free systems. Journal of virology 1992;66(10):6155-63.

A Taraboulos; K Jendroska; D Serban; S L Yang; S J DeArmond; S B Prusiner Regional mapping of prion proteins in brain. Proceedings of the National Academy of Sciences of the United States of America 1992;89(16):7620-4.

D R Borchelt; A Taraboulos; S B Prusiner Evidence for synthesis of scrapie prion proteins in the endocytic pathway. The Journal of biological chemistry 1992;267(23):16188-99.

A Taraboulos; A J Raeber; D R Borchelt; D Serban; S B Prusiner Synthesis and trafficking of prion proteins in cultured cells. Molecular biology of the cell 1992;3(8):851-63.

M R Scott; R Köhler; D Foster; S B Prusiner Chimeric prion protein expression in cultured cells and transgenic mice. Protein science : a publication of the Protein Society 1992;1(8):986-97.

R Hecker; A Taraboulos; M Scott; K M Pan; S L Yang; M Torchia; K Jendroska; S J DeArmond; S B Prusiner Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters. Genes & development 1992;6(7):1213-28.

Z Meiner; M Halimi; R D Polakiewicz; S B Prusiner; R Gabizon Presence of prion protein in peripheral tissues of Libyan Jews with Creutzfeldt-Jakob disease. Neurology 1992;42(7):1355-60.

N Stahl; M A Baldwin; R Hecker; K M Pan; A L Burlingame; S B Prusiner Glycosylinositol phospholipid anchors of the scrapie and cellular prion proteins contain sialic acid. Biochemistry 1992;31(21):5043-53.

H Büeler; M Fischer; Y Lang; H Bluethmann; H P Lipp; S J DeArmond; S B Prusiner; M Aguet; C Weissmann Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature 1992;356(6370):577-82.

K Hsiao; S R Dlouhy; M R Farlow; C Cass; M Da Costa; P M Conneally; M E Hodes; B Ghetti; S B Prusiner Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles. Nature genetics 1992;1(1):68-71.

S R Dlouhy; K Hsiao; M R Farlow; T Foroud; P M Conneally; P Johnson; S B Prusiner; M E Hodes; B Ghetti Linkage of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease to the prion protein gene. Nature genetics 1992;1(1):64-7.

K Kellings; N Meyer; C Mirenda; S B Prusiner; D Riesner Further analysis of nucleic acids in purified scrapie prion preparations by improved return refocusing gel electrophoresis. The Journal of general virology 1992;73 ( Pt 4)():1025-9.

H A Kretzschmar; P Kufer; G Riethmüller; S DeArmond; S B Prusiner; D Schiffer Prion protein mutation at codon 102 in an Italian family with Gerstmann-Sträussler-Scheinker syndrome. Neurology 1992;42(4):809-10.

S B Prusiner Molecular biology and genetics of neurodegenerative diseases caused by prions. Advances in virus research 1992;41():241-80.

M P McKinley; A Taraboulos; L Kenaga; D Serban; A Stieber; S J DeArmond; S B Prusiner; N Gonatas Ultrastructural localization of scrapie prion proteins in cytoplasmic vesicles of infected cultured cells. Laboratory investigation; a journal of technical methods and pathology 1991;65(6):622-30.

M Rogers; D Serban; T Gyuris; M Scott; T Torchia; S B Prusiner Epitope mapping of the Syrian hamster prion protein utilizing chimeric and mutant genes in a vaccinia virus expression system. Journal of immunology (Baltimore, Md. : 1950) 1991;147(10):3568-74.

N Stahl; S B Prusiner Prions and prion proteins. The FASEB journal : official publication of the Federation of American Societies for Experimental Biology 1991;5(13):2799-807.

N Stahl; M A Baldwin; S B Prusiner Electrospray mass spectrometry of the glycosylinositol phospholipid of the scrapie prion protein. Cell biology international reports 1991;15(9):853-62.

K Jendroska; F P Heinzel; M Torchia; L Stowring; H A Kretzschmar; A Kon; A Stern; S B Prusiner; S J DeArmond Proteinase-resistant prion protein accumulation in Syrian hamster brain correlates with regional pathology and scrapie infectivity. Neurology 1991;41(9):1482-90.

S B Prusiner; S J DeArmond Molecular biology and pathology of scrapie and the prion diseases of humans. Brain pathology (Zurich, Switzerland) 1991;1(4):297-310.

S B Prusiner Molecular biology of prion diseases. Science (New York, N.Y.) 1991;252(5012):1515-22.

K K Hsiao; C Cass; G D Schellenberg; T Bird; E Devine-Gage; H Wisniewski; S B Prusiner A prion protein variant in a family with the telencephalic form of Gerstmann-Sträussler-Scheinker syndrome. Neurology 1991;41(5):681-4.

S B Prusiner; D Westaway Infectious and genetic manifestations of prion diseases. Molecular plant-microbe interactions : MPMI 1991;4(3):226-33.

K Hsiao; Z Meiner; E Kahana; C Cass; I Kahana; D Avrahami; G Scarlato; O Abramsky; S B Prusiner; R Gabizon Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease. The New England journal of medicine 1991;324(16):1091-7.

S B Prusiner; M Torchia; D Westaway Molecular biology and genetics of prions–implications for sheep scrapie, ”mad cows” and the BSE epidemic. Historical background. The Cornell veterinarian 1991;81(2):85-101.

M P McKinley; R K Meyer; L Kenaga; F Rahbar; R Cotter; A Serban; S B Prusiner Scrapie prion rod formation in vitro requires both detergent extraction and limited proteolysis. Journal of virology 1991;65(3):1340-51.

F Tagliavini; F Prelli; J Ghiso; O Bugiani; D Serban; S B Prusiner; M R Farlow; B Ghetti; B Frangione Amyloid protein of Gerstmann-Sträussler-Scheinker disease (Indiana kindred) is an 11 kd fragment of prion protein with an N-terminal glycine at codon 58. The EMBO journal 1991;10(3):513-9.

G A Carlson; K Hsiao; B Oesch; D Westaway; S B Prusiner Genetics of prion infections. Trends in genetics : TIG 1991;7(2):61-5.

C J Epstein; D B Foster; S J DeArmond; S B Prusiner Acceleration of scrapie in trisomy 16—-diploid aggregation chimeras. Annals of neurology 1991;29(1):95-7.

N Meyer; V Rosenbaum; B Schmidt; K Gilles; C Mirenda; D Groth; S B Prusiner; D Riesner Search for a putative scrapie genome in purified prion fractions reveals a paucity of nucleic acids. The Journal of general virology 1991;72 ( Pt 1)():37-49.

M P McKinley; S B Prusiner Ultrastructural studies of prions. Current topics in microbiology and immunology 1991;172():75-91.

S B Prusiner Novel properties and biology of scrapie prions. Current topics in microbiology and immunology 1991;172():233-57.

B Oesch; D Westaway; S B Prusiner Prion protein genes: evolutionary and functional aspects. Current topics in microbiology and immunology 1991;172():109-24.

S B Prusiner Molecular biology of prions causing infectious and genetic encephalopathies of humans as well as scrapie of sheep and BSE of cattle. Developments in biological standardization 1991;75():55-74.

K Hsiao; M Scott; D Foster; S J DeArmond; D Groth; H Serban; S B Prusiner Spontaneous neurodegeneration in transgenic mice with prion protein codon 101 proline—-leucine substitution. Annals of the New York Academy of Sciences 1991;640():166-70.

K Hsiao; S B Prusiner Molecular genetics and transgenic model of Gertsmann-Sträussler-Scheinker disease. Alzheimer disease and associated disorders 1991;5(3):155-62.

S B Prusiner Molecular biology and transgenetics of prion diseases. Critical reviews in biochemistry and molecular biology 1991;26(5-6):397-438.

S B Prusiner Prion biology and diseases. Harvey lectures 1991;87():85-114.

K K Hsiao; M Scott; D Foster; D F Groth; S J DeArmond; S B Prusiner Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science (New York, N.Y.) 1990;250(4987):1587-90.

K Hsiao; S B Prusiner Inherited human prion diseases. Neurology 1990;40(12):1820-7.

S B Prusiner; M Scott; D Foster; K M Pan; D Groth; C Mirenda; M Torchia; S L Yang; D Serban; G A Carlson Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell 1990;63(4):673-86.

M A Baldwin; N Stahl; L G Reinders; B W Gibson; S B Prusiner; A L Burlingame Permethylation and tandem mass spectrometry of oligosaccharides having free hexosamine: analysis of the glycoinositol phospholipid anchor glycan from the scrapie prion protein. Analytical biochemistry 1990;191(1):174-82.

A Taraboulos; M Rogers; D R Borchelt; M P McKinley; M Scott; D Serban; S B Prusiner Acquisition of protease resistance by prion proteins in scrapie-infected cells does not require asparagine-linked glycosylation. Proceedings of the National Academy of Sciences of the United States of America 1990;87(21):8262-6.

A D Snow; T N Wight; D Nochlin; Y Koike; K Kimata; S J DeArmond; S B Prusiner Immunolocalization of heparan sulfate proteoglycans to the prion protein amyloid plaques of Gerstmann-Straussler syndrome, Creutzfeldt-Jakob disease and scrapie. Laboratory investigation; a journal of technical methods and pathology 1990;63(5):601-11.

S B Prusiner Novel structure and genetics of prions causing neurodegeneration in humans and animals. Biologicals : journal of the International Association of Biological Standardization 1990;18(4):247-62.

N Stahl; M A Baldwin; A L Burlingame; S B Prusiner Identification of glycoinositol phospholipid linked and truncated forms of the scrapie prion protein. Biochemistry 1990;29(38):8879-84.

M Rogers; A Taraboulos; M Scott; D Groth; S B Prusiner Intracellular accumulation of the cellular prion protein after mutagenesis of its Asn-linked glycosylation sites. Glycobiology 1990;1(1):101-9.

D Westaway; S B Prusiner Link between scrapie and BSE? Nature 1990;346(6280):113.

B Oesch; D B Teplow; N Stahl; D Serban; L E Hood; S B Prusiner Identification of cellular proteins binding to the scrapie prion protein. Biochemistry 1990;29(24):5848-55.

N Stahl; D R Borchelt; S B Prusiner Differential release of cellular and scrapie prion proteins from cellular membranes by phosphatidylinositol-specific phospholipase C. Biochemistry 1990;29(22):5405-12.

A Taraboulos; D Serban; S B Prusiner Scrapie prion proteins accumulate in the cytoplasm of persistently infected cultured cells. The Journal of cell biology 1990;110(6):2117-32.

C D Lopez; C S Yost; S B Prusiner; R M Myers; V R Lingappa Unusual topogenic sequence directs prion protein biogenesis. Science (New York, N.Y.) 1990;248(4952):226-9.

D H Lowenstein; D A Butler; D Westaway; M P McKinley; S J DeArmond; S B Prusiner Three hamster species with different scrapie incubation times and neuropathological features encode distinct prion proteins. Molecular and cellular biology 1990;10(3):1153-63.

D R Borchelt; M Scott; A Taraboulos; N Stahl; S B Prusiner Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells. The Journal of cell biology 1990;110(3):743-52.

K B Boylan; N Takahashi; D W Paty; A D Sadovnick; M Diamond; L E Hood; S B Prusiner DNA length polymorphism 5’ to the myelin basic protein gene is associated with multiple sclerosis. Annals of neurology 1990;27(3):291-7.

R Gabizon; S B Prusiner Prion liposomes. The Biochemical journal 1990;266(1):1-14.

C S Yost; C D Lopez; S B Prusiner; R M Myers; V R Lingappa Non-hydrophobic extracytoplasmic determinant of stop transfer in the prion protein. Nature 1990;343(6259):669-72.

S B Prusiner; S J DeArmond Prion diseases of the central nervous system. Monographs in pathology 1990;(32):86-122.

M P McKinley; F M Longo; J S Valletta; F Rahbar; R L Neve; S B Prusiner; W C Mobley Nerve growth factor induces gene expression of the prion protein and beta-amyloid protein precursor in the developing hamster central nervous system. Progress in brain research 1990;86():227-38.

D Serban; A Taraboulos; S J DeArmond; S B Prusiner Rapid detection of Creutzfeldt-Jakob disease and scrapie prion proteins. Neurology 1990;40(1):110-7.

K B Boylan; T M Ayres; B Popko; N Takahashi; L E Hood; S B Prusiner Repetitive DNA (TGGA)n 5’ to the human myelin basic protein gene: a new form of oligonucleotide repetitive sequence showing length polymorphism. Genomics 1990;6(1):16-22.

T Endo; D Groth; S B Prusiner; A Kobata Diversity of oligosaccharide structures linked to asparagines of the scrapie prion protein. Biochemistry 1989;28(21):8380-8.

G A Carlson; D Westaway; S J DeArmond; M Peterson-Torchia; S B Prusiner Primary structure of prion protein may modify scrapie isolate properties. Proceedings of the National Academy of Sciences of the United States of America 1989;86(19):7475-9.

M P McKinley; S J DeArmond; M Torchia; W C Mobley; S B Prusiner Acceleration of scrapie in neonatal Syrian hamsters. Neurology 1989;39(10):1319-24.

D Westaway; G A Carlson; S B Prusiner Unraveling prion diseases through molecular genetics. Trends in neurosciences 1989;12(6):221-7.

K Hsiao; H F Baker; T J Crow; M Poulter; F Owen; J D Terwilliger; D Westaway; J Ott; S B Prusiner Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome. Nature 1989;338(6213):342-5.

S B Prusiner Creutzfeldt-Jakob disease and scrapie prions. Alzheimer disease and associated disorders 1989;3(1-2):52-78.

F Owen; M Poulter; R Lofthouse; J Collinge; T J Crow; D Risby; H F Baker; R M Ridley; K Hsiao; S B Prusiner Insertion in prion protein gene in familial Creutzfeldt-Jakob disease. Lancet 1989;1(8628):51-2.

S J De Armond; M Gonzales; W C Mobley; A A Kon; A Stern; H Prusiner; S B Prusiner PrPSc in scrapie-infected hamster brain is spatially and temporally related to histopathology and infectivity titer. Progress in clinical and biological research 1989;317():601-18.

R Gabizon; M P McKinley; D Groth; D Westaway; S J DeArmond; G A Carlson; S B Prusiner Immunoaffinity purification and neutralization of scrapie prions. Progress in clinical and biological research 1989;317():583-600.

S B Prusiner Scrapie prions. Annual review of microbiology 1989;43():345-74.

A D Snow; R Kisilevsky; J Willmer; S B Prusiner; S J DeArmond Sulfated glycosaminoglycans in amyloid plaques of prion diseases. Acta neuropathologica 1989;77(4):337-42.

G A Carlson; P A Goodman; M Lovett; B A Taylor; S T Marshall; M Peterson-Torchia; D Westaway; S B Prusiner Genetics and polymorphism of the mouse prion gene complex: control of scrapie incubation time. Molecular and cellular biology 1988;8(12):5528-40.

W C Mobley; R L Neve; S B Prusiner; M P McKinley Nerve growth factor increases mRNA levels for the prion protein and the beta-amyloid protein precursor in developing hamster brain. Proceedings of the National Academy of Sciences of the United States of America 1988;85(24):9811-5.

G W Roberts; R Lofthouse; D Allsop; M Landon; M Kidd; S B Prusiner; T J Crow CNS amyloid proteins in neurodegenerative diseases. Neurology 1988;38(10):1534-40.

E Turk; D B Teplow; L E Hood; S B Prusiner Purification and properties of the cellular and scrapie hamster prion proteins. European journal of biochemistry / FEBS 1988;176(1):21-30.

R Gabizon; M P McKinley; D Groth; S B Prusiner Immunoaffinity purification and neutralization of scrapie prion infectivity. Proceedings of the National Academy of Sciences of the United States of America 1988;85(18):6617-21.

C G Bellinger-Kawahara; E Kempner; D Groth; R Gabizon; S B Prusiner Scrapie prion liposomes and rods exhibit target sizes of 55,000 Da. Virology 1988;164(2):537-41.

D A Butler; M R Scott; J M Bockman; D R Borchelt; A Taraboulos; K K Hsiao; D T Kingsbury; S B Prusiner Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins. Journal of virology 1988;62(5):1558-64.

R Gabizon; M P McKinley; D F Groth; L Kenaga; S B Prusiner Properties of scrapie prion protein liposomes. The Journal of biological chemistry 1988;263(10):4950-5.

M R Scott; D A Butler; D E Bredesen; M Wälchli; K K Hsiao; S B Prusiner Prion protein gene expression in cultured cells. Protein engineering 1988;2(1):69-76.

R A Barry; M T Vincent; S B Kent; L E Hood; S B Prusiner Characterization of prion proteins with monospecific antisera to synthetic peptides. Journal of immunology (Baltimore, Md. : 1950) 1988;140(4):1188-93.

B Oesch; D F Groth; S B Prusiner; C Weissmann Search for a scrapie-specific nucleic acid: a progress report. Ciba Foundation symposium 1988;135():209-23.

S B Prusiner Molecular structure, biology, and genetics of prions. Advances in virus research 1988;35():83-136.

G A Carlson; D Westaway; P A Goodman; M Peterson; S T Marshall; S B Prusiner Genetic control of prion incubation period in mice. Ciba Foundation symposium 1988;135():84-99.

S B Prusiner; N Stahl; S J DeArmond Novel mechanisms of degeneration of the central nervous system–prion structure and biology. Ciba Foundation symposium 1988;135():239-60.

R Gabizon; M P McKinley; S B Prusiner Properties of scrapie prion proteins in liposomes and amyloid rods. Ciba Foundation symposium 1988;135():182-96.

M P McKinley; V R Lingappa; S B Prusiner Developmental regulation of prion protein mRNA in brain. Ciba Foundation symposium 1988;135():101-16.

S B Prusiner Prions and neurodegenerative diseases. The New England journal of medicine 1987;317(25):1571-81.

B Hay; S B Prusiner; V R Lingappa Evidence for a secretory form of the cellular prion protein. Biochemistry 1987;26(25):8110-5.

M J Braun; M A Gonda; D G George; J F Bazan; R J Fletterick; S B Prusiner The burden of proof in linking AIDS to scrapie. Nature 1987;330(6148):525-6.

C A Wiley; P G Burrola; M J Buchmeier; M K Wooddell; R A Barry; S B Prusiner; P W Lampert Immuno-gold localization of prion filaments in scrapie-infected hamster brains. Laboratory investigation; a journal of technical methods and pathology 1987;57(6):646-56.

D Westaway; P A Goodman; C A Mirenda; M P McKinley; G A Carlson; S B Prusiner Distinct prion proteins in short and long scrapie incubation period mice. Cell 1987;51(4):651-62.

N Stahl; D R Borchelt; K Hsiao; S B Prusiner Scrapie prion protein contains a phosphatidylinositol glycolipid. Cell 1987;51(2):229-40.

C Bellinger-Kawahara; T O Diener; M P McKinley; D F Groth; D R Smith; S B Prusiner Purified scrapie prions resist inactivation by procedures that hydrolyze, modify, or shear nucleic acids. Virology 1987;160(1):271-4.

S J DeArmond; W C Mobley; D L DeMott; R A Barry; J H Beckstead; S B Prusiner Changes in the localization of brain prion proteins during scrapie infection. Neurology 1987;37(8):1271-80.

T Kitamoto; K Ogomori; J Tateishi; S B Prusiner Formic acid pretreatment enhances immunostaining of cerebral and systemic amyloids. Laboratory investigation; a journal of technical methods and pathology 1987;57(2):230-6.

R N Hogan; J R Baringer; S B Prusiner Scrapie infection diminishes spines and increases varicosities of dendrites in hamsters: a quantitative Golgi analysis. Journal of neuropathology and experimental neurology 1987;46(4):461-73.

J M Bockman; S B Prusiner; J Tateishi; D T Kingsbury Immunoblotting of Creutzfeldt-Jakob disease prion proteins: host species-specific epitopes. Annals of neurology 1987;21(6):589-95.

R Gabizon; M P McKinley; S B Prusiner Purified prion proteins and scrapie infectivity copartition into liposomes. Proceedings of the National Academy of Sciences of the United States of America 1987;84(12):4017-21.

M P McKinley; B Hay; V R Lingappa; I Lieberburg; S B Prusiner Developmental expression of prion protein gene in brain. Developmental biology 1987;121(1):105-10.

K B Boylan; N Takahashi; M Diamond; L E Hood; S B Prusiner DNA length polymorphism located 5’ to the human myelin basic protein gene. American journal of human genetics 1987;40(5):387-400.

S B Prusiner Prion diseases and central nervous system degeneration. Clinical research 1987;35(3):177-91.

S B Prusiner; S J DeArmond Prions causing nervous system degeneration. Laboratory investigation; a journal of technical methods and pathology 1987;56(4):349-63.

J Fernando Bazan; R J Fletterick; S B Prusiner AIDS virus and scrapie protein genes. Nature 1987;325(6105):581.

B Hay; R A Barry; I Lieberburg; S B Prusiner; V R Lingappa Biogenesis and transmembrane orientation of the cellular isoform of the scrapie prion protein [published errratum appears in Mol Cell Biol 1987 May;7(5):2035]. Molecular and cellular biology 1987;7(2):914-20.

J F Bazan; R J Fletterick; M P McKinley; S B Prusiner Predicted secondary structure and membrane topology of the scrapie prion protein. Protein engineering 1987;1(2):125-35.

S B Prusiner Prions causing degenerative neurological diseases. Annual review of medicine 1987;38():381-98.

S B Prusiner; R Gabizon; M P McKinley On the biology of prions. Acta neuropathologica 1987;72(4):299-314.

C Bellinger-Kawahara; J E Cleaver; T O Diener; S B Prusiner Purified scrapie prions resist inactivation by UV irradiation. Journal of virology 1987;61(1):159-66.

S B Prusiner Prions are novel infectious pathogens causing scrapie and Creutzfeldt-Jakob disease. BioEssays : news and reviews in molecular, cellular and developmental biology 1986;5(6):281-6.

G W Roberts; R Lofthouse; R Brown; T J Crow; R A Barry; S B Prusiner Prion-protein immunoreactivity in human transmissible dementias. The New England journal of medicine 1986;315(19):1231-3.

R B Crook; S B Prusiner Vasoactive intestinal peptide stimulates cyclic AMP metabolism in choroid plexus epithelial cells. Brain research 1986;384(1):138-44.

R A Barry; S B Prusiner Monoclonal antibodies to the cellular and scrapie prion proteins. The Journal of infectious diseases 1986;154(3):518-21.

G A Carlson; D T Kingsbury; P A Goodman; S Coleman; S T Marshall; S DeArmond; D Westaway; S B Prusiner Linkage of prion protein and scrapie incubation time genes. Cell 1986;46(4):503-11.

H A Kretzschmar; L E Stowring; D Westaway; W H Stubblebine; S B Prusiner; S J Dearmond Molecular cloning of a human prion protein cDNA. DNA (Mary Ann Liebert, Inc.) 1986;5(4):315-24.

T Kitamoto; J Tateishi; T Tashima; I Takeshita; R A Barry; S J DeArmond; S B Prusiner Amyloid plaques in Creutzfeldt-Jakob disease stain with prion protein antibodies. Annals of neurology 1986;20(2):204-8.

K Basler; B Oesch; M Scott; D Westaway; M Wälchli; D F Groth; M P McKinley; S B Prusiner; C Weissmann Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene. Cell 1986;46(3):417-28.

M P McKinley; M B Braunfeld; C G Bellinger; S B Prusiner Molecular characteristics of prion rods purified from scrapie-infected hamster brains. The Journal of infectious diseases 1986;154(1):110-20.

R A Barry; S B Kent; M P McKinley; R K Meyer; S J DeArmond; L E Hood; S B Prusiner Scrapie and cellular prion proteins share polypeptide epitopes. The Journal of infectious diseases 1986;153(5):848-54.

R K Meyer; M P McKinley; K A Bowman; M B Braunfeld; R A Barry; S B Prusiner Separation and properties of cellular and scrapie prion proteins. Proceedings of the National Academy of Sciences of the United States of America 1986;83(8):2310-4.

D Westaway; S B Prusiner Conservation of the cellular gene encoding the scrapie prion protein. Nucleic acids research 1986;14(5):2035-44.

R B Crook; M B Farber; S B Prusiner H2 histamine receptors on the epithelial cells of choroid plexus. Journal of neurochemistry 1986;46(2):489-93.

M P McKinley; S B Prusiner Biology and structure of scrapie prions. International review of neurobiology 1986;28():1-57.

R N Hogan; K A Bowman; J R Baringer; S B Prusiner Replication of scrapie prions in hamster eyes precedes retinal degeneration. Ophthalmic research 1986;18(4):230-5.

H A Kretzschmar; S B Prusiner; L E Stowring; S J DeArmond Scrapie prion proteins are synthesized in neurons. The American journal of pathology 1986;122(1):1-5.

S B Prusiner; S P Cochran; M P Alpers Transmission of scrapie in hamsters. The Journal of infectious diseases 1985;152(5):971-8.

M L Monteiro; R A Swanson; J R Coppeto; R A Cuneo; S J DeArmond; S B Prusiner A microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions. Neurology 1985;35(8):1113-21.

N Takahashi; A Roach; D B Teplow; S B Prusiner; L Hood Cloning and characterization of the myelin basic protein gene from mouse: one gene can encode both 14 kd and 18.5 kd MBPs by alternate use of exons. Cell 1985;42(1):139-48.

R A Barry; M P McKinley; P E Bendheim; G K Lewis; S J DeArmond; S B Prusiner Antibodies to the scrapie protein decorate prion rods. Journal of immunology (Baltimore, Md. : 1950) 1985;135(1):603-13.

S J DeArmond; M P McKinley; R A Barry; M B Braunfeld; J R McColloch; S B Prusiner Identification of prion amyloid filaments in scrapie-infected brain. Cell 1985;41(1):221-35.

D C Bolton; R K Meyer; S B Prusiner Scrapie PrP 27-30 is a sialoglycoprotein. Journal of virology 1985;53(2):596-606.

P E Bendheim; J M Bockman; M P McKinley; D T Kingsbury; S B Prusiner Scrapie and Creutzfeldt-Jakob disease prion proteins share physical properties and antigenic determinants. Proceedings of the National Academy of Sciences of the United States of America 1985;82(4):997-1001.

J M Bockman; D T Kingsbury; M P McKinley; P E Bendheim; S B Prusiner Creutzfeldt-Jakob disease prion proteins in human brains. The New England journal of medicine 1985;312(2):73-8.

S B Prusiner; R A Barry; M P McKinley; C G Bellinger; R K Meyer; S J DeArmond; D T Kingsbury Scrapie and Creutzfeldt-Jakob disease prions. Microbiological sciences 1985;2(2):33-9.

S B Prusiner; D T Kingsbury Prions–infectious pathogens causing the spongiform encephalopathies. CRC critical reviews in clinical neurobiology 1985;1(3):181-200.

S B Prusiner Scrapie prions, brain amyloid, and senile dementia. Current topics in cellular regulation 1985;26():79-95.

D C Bolton; M P McKinley; S B Prusiner Molecular characteristics of the major scrapie prion protein. Biochemistry 1984;23(25):5898-906.

S B Prusiner Prions. Scientific American 1984;251(4):50-9.

R P Friedland; S B Prusiner; W J Jagust; T F Budinger; R L Davis Bitemporal hypometabolism in Creutzfeldt-Jakob disease measured by positron emission tomography with [18F]-2-fluorodeoxyglucose. Journal of computer assisted tomography 1984;8(5):978-81.

P E Bendheim; R A Barry; S J DeArmond; D P Stites; S B Prusiner Antibodies to a scrapie prion protein. Nature 1984;310(5976):418-21.

S B Prusiner; D F Groth; D C Bolton; S B Kent; L E Hood Purification and structural studies of a major scrapie prion protein. Cell 1984;38(1):127-34.

S B Prusiner Some speculations about prions, amyloid, and Alzheimer’s disease. The New England journal of medicine 1984;310(10):661-3.

R B Crook; M B Farber; S B Prusiner Hormones and neurotransmitters control cyclic AMP metabolism in choroid plexus epithelial cells. Journal of neurochemistry 1984;42(2):340-50.

S B Prusiner Prions: novel infectious pathogens. Advances in virus research 1984;29():1-56.

S B Prusiner; M P McKinley; K A Bowman; D C Bolton; P E Bendheim; D F Groth; G G Glenner Scrapie prions aggregate to form amyloid-like birefringent rods. Cell 1983;35(2 Pt 1):349-58.

R N Hogan; D T Kingsbury; J R Baringer; S B Prusiner Retinal degeneration in experimental Creutzfeldt-Jakob disease. Laboratory investigation; a journal of technical methods and pathology 1983;49(6):708-15.

M P McKinley; D C Bolton; S B Prusiner A protease-resistant protein is a structural component of the scrapie prion. Cell 1983;35(1):57-62.

A Roach; K Boylan; S Horvath; S B Prusiner; L E Hood Characterization of cloned cDNA representing rat myelin basic protein: absence of expression in brain of shiverer mutant mice. Cell 1983;34(3):799-806.

J R Baringer; K A Bowman; S B Prusiner Replication of the scrapie agent in hamster brain precedes neuronal vacuolation. Journal of neuropathology and experimental neurology 1983;42(5):539-47.

D T Kingsbury; K C Kasper; D P Stites; J D Watson; R N Hogan; S B Prusiner Genetic control of scrapie and Creutzfeldt-Jakob disease in mice. Journal of immunology (Baltimore, Md. : 1950) 1983;131(1):491-6.

M P McKinley; F R Masiarz; S T Isaacs; J E Hearst; S B Prusiner Resistance of the scrapie agent to inactivation by psoralens. Photochemistry and photobiology 1983;37(5):539-45.

D C Bolton; M P McKinley; S B Prusiner Identification of a protein that purifies with the scrapie prion. Science (New York, N.Y.) 1982;218(4579):1309-11.

S B Prusiner; D C Bolton; D F Groth; K A Bowman; S P Cochran; M P McKinley Further purification and characterization of scrapie prions. Biochemistry 1982;21(26):6942-50.

K C Kasper; D P Stites; K A Bowman; H Panitch; S B Prusiner Immunological studies of scrapie infection. Journal of neuroimmunology 1982;3(3):187-201.

T O Diener; M P McKinley; S B Prusiner Viroids and prions. Proceedings of the National Academy of Sciences of the United States of America 1982;79(17):5220-4.

S B Prusiner Research on scrapie. Lancet 1982;2(8296):494-5.

S B Prusiner; C Gajdusek; M P Alpers Kuru with incubation periods exceeding two decades. Annals of neurology 1982;12(1):1-9.

S B Prusiner Novel proteinaceous infectious particles cause scrapie. Science (New York, N.Y.) 1982;216(4542):136-44.

S B Prusiner; S P Cochran; D F Groth; D E Downey; K A Bowman; H M Martinez Measurement of the scrapie agent using an incubation time interval assay. Annals of neurology 1982;11(4):353-8.

M P McKinley; F R Masiarz; S B Prusiner Reversible chemical modification of the scrapie agent. Science (New York, N.Y.) 1981;214(4526):1259-61.

S B Prusiner; M P McKinley; D F Groth; K A Bowman; N I Mock; S P Cochran; F R Masiarz Scrapie agent contains a hydrophobic protein. Proceedings of the National Academy of Sciences of the United States of America 1981;78(11):6675-9.

R B Crook; H Kasagami; S B Prusiner Culture and characterization of epithelial cells from bovine choroid plexus. Journal of neurochemistry 1981;37(4):845-54.

R D Mamelok; D R Macrae; L Z Benet; S B Prusiner Membrane populations of bovine choroid plexus: separation by density gradient centrifugation in modified colloidal silica. Journal of neurochemistry 1981;37(3):768-74.

S B Prusiner; D F Groth; M P McKinley; S P Cochran; K A Bowman; K C Kasper Thiocyanate and hydroxyl ions inactivate the scrapie agent. Proceedings of the National Academy of Sciences of the United States of America 1981;78(7):4606-10.

J R Baringer; S B Prusiner; J S Wong Scrapie-associated particles in postsynaptic processes. Further ultrastructural studies. Journal of neuropathology and experimental neurology 1981;40(3):281-8.

R D Mamelok; D R Macrae; K Hittelman; J P Hoefer; S B Prusiner Kinetics of D-glucose transport into renal membrane vesicles: measurements using a vacuum manifold apparatus. Journal of biochemical and biophysical methods 1981;4(3-4):147-53.

S B Prusiner; S P Cochran; D E Downey; D F Groth Determination of scrapie agent titer from incubation period measurements in hamsters. Advances in experimental medicine and biology 1981;134():385-99.

S B Prusiner Disorders of glutamate metabolism and neurological dysfunction. Annual review of medicine 1981;32():521-42.

R N Hogan; J R Baringer; S B Prusiner Progressive retinal degeneration in scrapie-infected hamsters: a light and electron microscopic analysis. Laboratory investigation; a journal of technical methods and pathology 1981;44(1):34-42.

K C Kasper; K Bowman; D P Stites; S B Prusiner Toward development of assays for scrapie-specific antibodies. Advances in experimental medicine and biology 1981;134():401-13.

W J Hadlow; S B Prusiner; R C Kennedy; R E Race Brain tissue from persons dying of Creutzfeldt-Jakob disease causes scrapie-like encephalopathy in goats. Annals of neurology 1980;8(6):628-32.

S B Prusiner; D F Groth; S P Cochran; M P McKinley; F R Masiarz Gel electrophoresis and glass permeation chromatography of the hamster scrapie agent after enzymatic digestion and detergent extraction. Biochemistry 1980;19(21):4892-8.

S B Prusiner; D F Groth; S P Cochran; F R Masiarz; M P McKinley; H M Martinez Molecular properties, partial purification, and assay by incubation period measurements of the hamster scrapie agent. Biochemistry 1980;19(21):4883-91.

S B Prusiner; D E Garfin; S P Cochran; M P McKinley; D F Groth; W J Hadlow; R E Race; C M Eklund Experimental scrapie in the mouse: electrophoretic and sedimentation properties of the partially purified agent. Journal of neurochemistry 1980;35(3):574-82.

M A Chatigny; S B Prusiner Biohazards of investigations on the transmissible spongiform encephalopathies. Reviews of infectious diseases 1980;2(5):713-24.

R D Mamelok; D F Groth; S B Prusiner Separation of membrane-bound gamma-glutamyl transpeptidase from brush border transport and enzyme activities. Biochemistry 1980;19(11):2367-73.

S B Prusiner; D F Groth; C Bildstein; F R Masiarz; M P McKinley; S P Cochran Electrophoretic properties of the scrapie agent in agarose gels. Proceedings of the National Academy of Sciences of the United States of America 1980;77(5):2984-8.

S B Prusiner; S P Cochran; J R Baringer; D Groth; F Masiarz; M McKinley; C Bildstein; D Garfin; W J Hadlow; R E Race; et al. Slow viruses: molecular properties of the agents causing scrapie in mice and hamsters. Progress in clinical and biological research 1980;39():73-89.

M A Chatigny; S Dunn; K Ishimaru; J A Eagleson; S B Prusiner Evaluation of a class III biological safety cabinet for enclosure of an ultracentrifuge. Applied and environmental microbiology 1979;38(5):934-9.

S B Prusiner; W J Hadlow; C M Eklund; R E Race; S P Cochran Sedimentation characteristics of the scrapie agent from murine spleen and brain. Biochemistry 1978;17(23):4987-92.

S B Prusiner; W J Hadlow; D E Garfin; S P Cochran; J R Baringer; R E Race; C M Eklund Partial purification and evidence for multiple molecular forms of the scrapie agent. Biochemistry 1978;17(23):4993-9.

K Hittelman; R D Mamelok; S B Prusiner Preservation by freezing of glucose and alanine transport into kidney membrane vesicles. Analytical biochemistry 1978;89(2):324-31.

J R Baringer; S B Prusiner Experimental scrapie in mice: ultrastructural observations. Annals of neurology 1978;4(3):205-11.

D E Garfin; D P Stites; J D Perlman; S P Cochran; S B Prusiner Mitogen stimulation of splenocytes from mice infected with scrapie agent. The Journal of infectious diseases 1978;138(3):396-400.

D E Garfin; D P Stites; L A Zitnik; S B Prusiner Suppression of polyclonal B cell activation in scrapie-infected C3H/HeJ mice. Journal of immunology (Baltimore, Md. : 1950) 1978;120(6):1986-90.

P E Prusiner; S B Prusiner Modulation of gamma-glutamyl transpeptidase activity from bovine choroid plexus. Journal of neurochemistry 1978;30(6):1261-7.

P E Prusiner; S B Prusiner Partial purification and kinetics of gamma-glutamyl transpeptidase from bovine choroid plexus. Journal of neurochemistry 1978;30(6):1253-9.

S B Prusiner An approach to the isolation of biological particles using sedimentation analysis. The Journal of biological chemistry 1978;253(3):916-21.

S B Prusiner; D E Garfin; S P Cochran; J R Baringer; W J Hadlow; C M Eklund; R E Race Evidence for hydrophobic domains on the surface of the scrapie agent. Transactions of the American Neurological Association 1978;103():62-4.

S B Prusiner; W J Hadlow; C M Eklund; R E Race Sedimentation properties of the scrapie agent. Proceedings of the National Academy of Sciences of the United States of America 1977;74(10):4656-60.

G Kirk; S B Prusiner Comparative studies on membranes from bovine choroid plexus and rat kidney cortex. Life sciences 1977;21(6):833-40.

N H Raskin; S Prusiner Carotidynia. Neurology 1977;27(1):43-6.

S Prusiner; C W Doak; G Kirk A novel mechanism for group translocation: substrate-product reutilization by gamma-glutamyl transpeptidase in peptide and amino acid transport. Journal of cellular physiology 1976;89(4):853-63.

S Prusiner; E R Stadtman Regulation of glutaminase B in Escherichia coli. III. Control by nucleotides and divalent cations. The Journal of biological chemistry 1976;251(11):3463-9.

S Prusiner; E R Stadtman Regulation of glutaminase B in Escherichia coli. II. Modulaltion of activity by carbosylate and borate ions. The Journal of biological chemistry 1976;251(11):3457-62.

S Prusiner; J N Davis; E R Stadtman Regulation of glutaminase B in Escherichia coli. I. Purification, properties, and cold lability. The Journal of biological chemistry 1976;251(11):3447-56.

S Prusiner Regulation of glutaminase levels in Escherichia coli. Journal of bacteriology 1975;123(3):992-9.

T F Deuel; S Prusiner Regulation of glutamine synthetase from Bacillus subtilis by divalent cations, feedback inhibitors, and L-glutamine. The Journal of biological chemistry 1974;249(1):257-64.

J N Davis; S Prusiner Stain for glutaminase activity. Analytical biochemistry 1973;54(1):272-5.

S Prusiner; R E Miller; R C Valentine Adenosine 3’:5’-cyclic monophosphate control of the enzymes of glutamine metabolism in Escherichia coli. Proceedings of the National Academy of Sciences of the United States of America 1972;69(10):2922-6.

S Prusiner; E R Stadtman On the regulation of glutaminase in E. coli: metabolite control. Biochemical and biophysical research communications 1971;45(6):1474-81.

S B Prusiner; L S Milner; C W Long; M L Myers Vacuum manifold for rapid assay of enzymes using radioactive tracers and ion exchange chromatography. The Review of scientific instruments 1971;42(4):493-4.

S Prusiner; L Milner A rapid radioactive assay for glutamine synthetase, glutaminase, asparagine synthetase, and asparaginase. Analytical biochemistry 1970;37(2):429-38.

O Lindberg; S B Prusiner; B Cannon; T M Ching; R H Eisenhardt Metabolic control in isolated brown fat cells. Lipids 1970;5(2):204-9.

S Prusiner Spectroscopic evidence for the control of respiration prior to phosphorylation in hamster brown fat cells. The Journal of biological chemistry 1970;245(2):382-9.

H J Reulen; U Steude; W Brendel; C Hilber; S Prusiner [Energetic disturbance of cation transport as a cause of intracellular brain edema]. Acta neurochirurgica 1970;22(2):129-66.

J R Williamson; S Prusiner; M S Olson; M Fukami Control of metabolism in brown adipose tissue. Lipids 1970;5(1):1-14.

S Prusiner; S K Wolfson Hypothermic protection against cerebral edema of ischemia. Archives of neurology 1968;19(6):623-7.

S B Prusiner; B Cannon; T M Ching; O Lindberg Oxidative metabolism in cells isolated from brown adipose tissue. 2. Catecholamine regulated respiratory control. European journal of biochemistry / FEBS 1968;7(1):51-7.

S Prusiner; M Poe Thermodynamic cosiderations of mammalian thermogenesis. Nature 1968;220(5164):235-7.

S B Prusiner; B Cannon; O Lindberg Oxidative metabolism in cells isolated from brown adipose tissue. 1. Catecholamine and fatty acid stimulation of respiration. European journal of biochemistry / FEBS 1968;6(1):15-22.

S B Prusiner; R H Eisenhardt; E Rylander; O Lindberg The regulation of oxidative metabolism of isolated brown fat cells. Biochemical and biophysical research communications 1968;30(5):508-15.

S Prusiner; J R Williamson; B Chance; B M Paddle Pyridine nucleotide changes during thermogenesis in brown fat tissue in vivo. Archives of biochemistry and biophysics 1968;123(2):368-77.

S B Prusiner; P A Moskovitz; S K Wolfson Relationship of acidemia to cerebral edema. Archives of surgery (Chicago, Ill. : 1960) 1965;91(6):902-5.



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    Institute for Neurodegenerative Diseases, University of California, San Francisco