Stanley Prusiner, MD

Director, Institute for Neurodegenerative Diseases
Professor, Department of Neurology
Neurology
+1 415 476-4482

Stanley B. Prusiner, MD, is a professor of neurology and biochemistry and director of the Institute for Neurodegenerative Diseases at UCSF. While at the university, Dr. Prusiner discovered an unprecedented class of pathogens that he named prions. Prions are infectious proteins that cause neurodegenerative diseases in animals and humans.

Dr. Prusiner’s contributions to scientific research have been internationally recognized with numerous prizes including the Richard Lounsbery Award, the Albert Lasker Award and the Nobel Prize.

Editor of 11 books and more than 500 research articles, Dr. Prusiner holds 50 issued or allowed United States patents, all of which are assigned to the University of California.

He is a member of the National Academy of Sciences, the Institute of Medicine, the American Academy of Arts and Sciences and the American Philosophical Society, and is a foreign member of the Royal Society, London

Dr. Prusiner received his undergraduate and medical training at the University of Pennsylvania and his postgraduate clinical training at UCSF.

Publications: 

Stacked binding of a PET ligand to Alzheimer's tau paired helical filaments.

Nature communications

Merz GE, Chalkley MJ, Tan SK, Tse E, Lee J, Prusiner SB, Paras NA, DeGrado WF, Southworth DR

Guam ALS-PDC is a distinct double-prion disorder featuring both tau and A� prions.

Proceedings of the National Academy of Sciences of the United States of America

Condello C, Ayers JI, Dalgard CL, Garcia Garcia MM, Rivera BM, Seeley WW, Perl DP, Prusiner SB

A� and Tau Prions Causing Alzheimer's Disease.

Methods in molecular biology (Clifton, N.J.)

Condello C, Merz GE, Aoyagi A, DeGrado WF, Prusiner SB

Aß and tau prions feature in the neuropathogenesis of Down syndrome.

Proceedings of the National Academy of Sciences of the United States of America

Condello C, Maxwell AM, Castillo E, Aoyagi A, Graff C, Ingelsson M, Lannfelt L, Bird TD, Keene CD, Seeley WW, Perl DP, Head E, Prusiner SB

Multiple system atrophy prions transmit neurological disease to mice expressing wild-type human a-synuclein.

Acta neuropathologica

Holec SAM, Lee J, Oehler A, Ooi FK, Mordes DA, Olson SH, Prusiner SB, Woerman AL

Trans-channel fluorescence learning improves high-content screening for Alzheimer's disease therapeutics.

Nature machine intelligence

Wong DR, Conrad J, Johnson N, Ayers J, Laeremans A, Lee JC, Lee J, Prusiner SB, Bandyopadhyay S, Butte AJ, Paras NA, Keiser MJ

Different a-synuclein prion strains cause dementia with Lewy bodies and multiple system atrophy.

Proceedings of the National Academy of Sciences of the United States of America

Ayers JI, Lee J, Monteiro O, Woerman AL, Lazar AA, Condello C, Paras NA, Prusiner SB

Prion biology: implications for Alzheimer's disease therapeutics.

The Lancet. Neurology

Condello C, DeGrado WF, Prusiner SB

Expanding spectrum of prion diseases.

Emerging topics in life sciences

Ayers JI, Paras NA, Prusiner SB

Prion protein - mediator of toxicity in multiple proteinopathies.

Nature reviews. Neurology

Ayers JI, Prusiner SB

Kinetics of a-synuclein prions preceding neuropathological inclusions in multiple system atrophy.

PLoS pathogens

Woerman AL, Patel S, Kazmi SA, Oehler A, Lee J, Mordes DA, Olson SH, Prusiner SB

Discovery of 4-Piperazine Isoquinoline Derivatives as Potent and Brain-Permeable Tau Prion Inhibitors with CDK8 Activity.

ACS medicinal chemistry letters

Grandjean JM, Jiu AY, West JW, Aoyagi A, Droege DG, Elepano M, Hirasawa M, Hirouchi M, Murakami R, Lee J, Sasaki K, Hirano S, Ohyama T, Tang BC, Vaz RJ, Inoue M, Olson SH, Prusiner SB, Conrad J, Paras NA

Replication of multiple system atrophy prions in primary astrocyte cultures from transgenic mice expressing human a-synuclein.

Acta neuropathologica communications

Krejciova Z, Carlson GA, Giles K, Prusiner SB

Aß and tau prion-like activities decline with longevity in the Alzheimer's disease human brain.

Science translational medicine

Aoyagi A, Condello C, Stöhr J, Yue W, Rivera BM, Lee JC, Woerman AL, Halliday G, van Duinen S, Ingelsson M, Lannfelt L, Graff C, Bird TD, Keene CD, Seeley WW, DeGrado WF, Prusiner SB

Multiple system atrophy prions retain strain specificity after serial propagation in two different Tg(SNCA*A53T) mouse lines.

Acta neuropathologica

Woerman AL, Oehler A, Kazmi SA, Lee J, Halliday GM, Middleton LT, Gentleman SM, Mordes DA, Spina S, Grinberg LT, Olson SH, Prusiner SB

a-Synuclein: Multiple System Atrophy Prions.

Cold Spring Harbor perspectives in medicine

Woerman AL, Watts JC, Aoyagi A, Giles K, Middleton LT, Prusiner SB

ß-Amyloid Prions and the Pathobiology of Alzheimer's Disease.

Cold Spring Harbor perspectives in medicine

Watts JC, Prusiner SB

A long-lived Aß oligomer resistant to fibrillization.

Biopolymers

Nick M, Wu Y, Schmidt NW, Prusiner SB, Stöhr J, DeGrado WF

Structural heterogeneity and intersubject variability of Aß in familial and sporadic Alzheimer's disease.

Proceedings of the National Academy of Sciences of the United States of America

Condello C, Lemmin T, Stöhr J, Nick M, Wu Y, Maxwell AM, Watts JC, Caro CD, Oehler A, Keene CD, Bird TD, van Duinen SG, Lannfelt L, Ingelsson M, Graff C, Giles K, DeGrado WF, Prusiner SB

Familial Parkinson's point mutation abolishes multiple system atrophy prion replication.

Proceedings of the National Academy of Sciences of the United States of America

Woerman AL, Kazmi SA, Patel S, Aoyagi A, Oehler A, Widjaja K, Mordes DA, Olson SH, Prusiner SB

Evidence for sortilin modulating regional accumulation of human tau prions in transgenic mice.

Proceedings of the National Academy of Sciences of the United States of America

Johnson NR, Condello C, Guan S, Oehler A, Becker J, Gavidia M, Carlson GA, Giles K, Prusiner SB

Kinetics of Human Mutant Tau Prion Formation in the Brains of 2 Transgenic Mouse Lines.

JAMA neurology

Woerman AL, Patel S, Kazmi SA, Oehler A, Freyman Y, Espiritu L, Cotter R, Castaneda JA, Olson SH, Prusiner SB

A novel vector for transgenesis in the rat CNS.

Acta neuropathologica communications

Lopez TP, Giles K, Dugger BN, Oehler A, Condello C, Krejciova Z, Castaneda JA, Carlson GA, Prusiner SB

Experimental Models of Inherited PrP Prion Diseases.

Cold Spring Harbor perspectives in medicine

Watts JC, Prusiner SB

MSA prions exhibit remarkable stability and resistance to inactivation.

Acta neuropathologica

Woerman AL, Kazmi SA, Patel S, Freyman Y, Oehler A, Aoyagi A, Mordes DA, Halliday GM, Middleton LT, Gentleman SM, Olson SH, Prusiner SB

Bioassays and Inactivation of Prions.

Cold Spring Harbor perspectives in biology

Giles K, Woerman AL, Berry DB, Prusiner SB

A 31-residue peptide induces aggregation of tau's microtubule-binding region in cells.

Nature chemistry

Stöhr J, Wu H, Nick M, Wu Y, Bhate M, Condello C, Johnson N, Rodgers J, Lemmin T, Acharya S, Becker J, Robinson K, Kelly MJS, Gai F, Stubbs G, Prusiner SB, DeGrado WF

Developing Therapeutics for PrP Prion Diseases.

Cold Spring Harbor perspectives in medicine

Giles K, Olson SH, Prusiner SB

Tau prions from Alzheimer's disease and chronic traumatic encephalopathy patients propagate in cultured cells.

Proceedings of the National Academy of Sciences of the United States of America

Woerman AL, Aoyagi A, Patel S, Kazmi SA, Lobach I, Grinberg LT, McKee AC, Seeley WW, Olson SH, Prusiner SB

Guinea Pig Prion Protein Supports Rapid Propagation of Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease Prions.

Journal of virology

Watts JC, Giles K, Saltzberg DJ, Dugger BN, Patel S, Oehler A, Bhardwaj S, Sali A, Prusiner SB

Structural Polymorphism of Alzheimer's ß-Amyloid Fibrils as Controlled by an E22 Switch: A Solid-State NMR Study.

Journal of the American Chemical Society

Elkins MR, Wang T, Nick M, Jo H, Lemmin T, Prusiner SB, DeGrado WF, Stöhr J, Hong M

FoxO3 regulates neuronal reprogramming of cells from postnatal and aging mice.

Proceedings of the National Academy of Sciences of the United States of America

Ahlenius H, Chanda S, Webb AE, Yousif I, Karmazin J, Prusiner SB, Brunet A, Südhof TC, Wernig M

Towards authentic transgenic mouse models of heritable PrP prion diseases.

Acta neuropathologica

Watts JC, Giles K, Bourkas ME, Patel S, Oehler A, Gavidia M, Bhardwaj S, Lee J, Prusiner SB

Optimization of Aryl Amides that Extend Survival in Prion-Infected Mice.

The Journal of pharmacology and experimental therapeutics

Giles K, Berry DB, Condello C, Dugger BN, Li Z, Oehler A, Bhardwaj S, Elepano M, Guan S, Silber BM, Olson SH, Prusiner SB

Evidence for a-synuclein prions causing multiple system atrophy in humans with parkinsonism.

Proceedings of the National Academy of Sciences of the United States of America

Prusiner SB, Woerman AL, Mordes DA, Watts JC, Rampersaud R, Berry DB, Patel S, Oehler A, Lowe JK, Kravitz SN, Geschwind DH, Glidden DV, Halliday GM, Middleton LT, Gentleman SM, Grinberg LT, Giles K

Modulation of Creutzfeldt-Jakob disease prion propagation by the A224V mutation.

Annals of neurology

Watts JC, Giles K, Serban A, Patel S, Oehler A, Bhardwaj S, Guan S, Greicius MD, Miller BL, DeArmond SJ, Geschwind MD, Prusiner SB

Prion Protein-Antibody Complexes Characterized by Chromatography-Coupled Small-Angle X-Ray Scattering.

Biophysical journal

Carter L, Kim SJ, Schneidman-Duhovny D, Stöhr J, Poncet-Montange G, Weiss TM, Tsuruta H, Prusiner SB, Sali A

Propagation of prions causing synucleinopathies in cultured cells.

Proceedings of the National Academy of Sciences of the United States of America

Woerman AL, Stöhr J, Aoyagi A, Rampersaud R, Krejciova Z, Watts JC, Ohyama T, Patel S, Widjaja K, Oehler A, Sanders DW, Diamond MI, Seeley WW, Middleton LT, Gentleman SM, Mordes DA, Südhof TC, Giles K, Prusiner SB

Different 2-Aminothiazole Therapeutics Produce Distinct Patterns of Scrapie Prion Neuropathology in Mouse Brains.

The Journal of pharmacology and experimental therapeutics

Giles K, Berry DB, Condello C, Hawley RC, Gallardo-Godoy A, Bryant C, Oehler A, Elepano M, Bhardwaj S, Patel S, Silber BM, Guan S, DeArmond SJ, Renslo AR, Prusiner SB

Use of a 2-aminothiazole to Treat Chronic Wasting Disease in Transgenic Mice.

The Journal of infectious diseases

Berry D, Giles K, Oehler A, Bhardwaj S, DeArmond SJ, Prusiner SB

Structural studies of truncated forms of the prion protein PrP.

Biophysical journal

Wan W, Wille H, Stöhr J, Kendall A, Bian W, McDonald M, Tiggelaar S, Watts JC, Prusiner SB, Stubbs G

Mechanism of scrapie prion precipitation with phosphotungstate anions.

ACS chemical biology

Levine DJ, Stöhr J, Falese LE, Ollesch J, Wille H, Prusiner SB, Long JR

Distinct synthetic Aß prion strains producing different amyloid deposits in bigenic mice.

Proceedings of the National Academy of Sciences of the United States of America

Stöhr J, Condello C, Watts JC, Bloch L, Oehler A, Nick M, DeArmond SJ, Giles K, DeGrado WF, Prusiner SB

Serial propagation of distinct strains of Aß prions from Alzheimer's disease patients.

Proceedings of the National Academy of Sciences of the United States of America

Watts JC, Condello C, Stöhr J, Oehler A, Lee J, DeArmond SJ, Lannfelt L, Ingelsson M, Giles K, Prusiner SB

Mouse models for studying the formation and propagation of prions.

The Journal of biological chemistry

Watts JC, Prusiner SB

Evidence that bank vole PrP is a universal acceptor for prions.

PLoS pathogens

Watts JC, Giles K, Patel S, Oehler A, DeArmond SJ, Prusiner SB

Novel compounds lowering the cellular isoform of the human prion protein in cultured human cells.

Bioorganic & medicinal chemistry

Silber BM, Gever JR, Rao S, Li Z, Renslo AR, Widjaja K, Wong C, Giles K, Freyman Y, Elepano M, Irwin JJ, Jacobson MP, Prusiner SB

Transmission of multiple system atrophy prions to transgenic mice.

Proceedings of the National Academy of Sciences of the United States of America

Watts JC, Giles K, Oehler A, Middleton L, Dexter DT, Gentleman SM, DeArmond SJ, Prusiner SB

Drug resistance confounding prion therapeutics.

Proceedings of the National Academy of Sciences of the United States of America

Berry DB, Lu D, Geva M, Watts JC, Bhardwaj S, Oehler A, Renslo AR, DeArmond SJ, Prusiner SB, Giles K

Quinacrine treatment trial for sporadic Creutzfeldt-Jakob disease.

Neurology

Geschwind MD, Kuo AL, Wong KS, Haman A, Devereux G, Raudabaugh BJ, Johnson DY, Torres-Chae CC, Finley R, Garcia P, Thai JN, Cheng HQ, Neuhaus JM, Forner SA, Duncan JL, Possin KL, Dearmond SJ, Prusiner SB, Miller BL

Antiprion compounds that reduce PrP(Sc) levels in dividing and stationary-phase cells.

Bioorganic & medicinal chemistry

Silber BM, Gever JR, Li Z, Gallardo-Godoy A, Renslo AR, Widjaja K, Irwin JJ, Rao S, Jacobson MP, Ghaemmaghami S, Prusiner SB

A high-throughput screening assay for determining cellular levels of total tau protein.

Current Alzheimer research

Dehdashti SJ, Zheng W, Gever JR, Wilhelm R, Nguyen DT, Sittampalam G, McKew JC, Austin CP, Prusiner SB

Biaryl amides and hydrazones as therapeutics for prion disease in transgenic mice.

The Journal of pharmacology and experimental therapeutics

Lu D, Giles K, Li Z, Rao S, Dolghih E, Gever JR, Geva M, Elepano ML, Oehler A, Bryant C, Renslo AR, Jacobson MP, Dearmond SJ, Silber BM, Prusiner SB

Optimization of Arylamides as Novel, Potent and Brain-penetrant Antiprion Lead Compounds.

ACS medicinal chemistry letters

Li Z, Rao S, Gever JR, Widjaja K, Prusiner SB, Silber BM

Discovery and Preliminary SAR of Arylpiperazines as Novel, Brainpenetrant Antiprion Compounds.

ACS medicinal chemistry letters

Li Z, Gever J, Rao S, Widjaja K, Prusiner SB, Silber BM

2-Aminothiazoles with improved pharmacotherapeutic properties for treatment of prion disease.

ChemMedChem

Li Z, Silber BM, Rao S, Gever JR, Bryant C, Gallardo-Godoy A, Dolghih E, Widjaja K, Elepano M, Jacobson MP, Prusiner SB, Renslo AR

Convergent replication of mouse synthetic prion strains.

The American journal of pathology

Ghaemmaghami S, Colby DW, Nguyen HO, Hayashi S, Oehler A, DeArmond SJ, Prusiner SB

Pharmacokinetics and metabolism of 2-aminothiazoles with antiprion activity in mice.

Pharmaceutical research

Silber BM, Rao S, Fife KL, Gallardo-Godoy A, Renslo AR, Dalvie DK, Giles K, Freyman Y, Elepano M, Gever JR, Li Z, Jacobson MP, Huang Y, Benet LZ, Prusiner SB

Biology and genetics of prions causing neurodegeneration.

Annual review of genetics

Prusiner SB

Chimeric elk/mouse prion proteins in transgenic mice.

The Journal of general virology

Tamgüney G, Giles K, Oehler A, Johnson NL, DeArmond SJ, Prusiner SB

Pharmacokinetics of quinacrine efflux from mouse brain via the P-glycoprotein efflux transporter.

PloS one

Ahn M, Ghaemmaghami S, Huang Y, Phuan PW, May BC, Giles K, DeArmond SJ, Prusiner SB

Purified and synthetic Alzheimer's amyloid beta (Aß) prions.

Proceedings of the National Academy of Sciences of the United States of America

Stöhr J, Watts JC, Mensinger ZL, Oehler A, Grillo SK, DeArmond SJ, Prusiner SB, Giles K

Degradation of fungal prion HET-s(218-289) induces formation of a generic amyloid fold.

Biophysical journal

Wan W, Wille H, Stöhr J, Baxa U, Prusiner SB, Stubbs G

Compartment modeling for mammalian protein turnover studies by stable isotope metabolic labeling.

Analytical chemistry

Guan S, Price JC, Ghaemmaghami S, Prusiner SB, Burlingame AL

Identification of I137M and other mutations that modulate incubation periods for two human prion strains.

Journal of virology

Giles K, De Nicola GF, Patel S, Glidden DV, Korth C, Oehler A, DeArmond SJ, Prusiner SB

Spontaneous generation of rapidly transmissible prions in transgenic mice expressing wild-type bank vole prion protein.

Proceedings of the National Academy of Sciences of the United States of America

Watts JC, Giles K, Stöhr J, Oehler A, Bhardwaj S, Grillo SK, Patel S, DeArmond SJ, Prusiner SB

Intracerebral Infusion of Antisense Oligonucleotides Into Prion-infected Mice.

Molecular therapy. Nucleic acids

Nazor Friberg K, Hung G, Wancewicz E, Giles K, Black C, Freier S, Bennett F, Dearmond SJ, Freyman Y, Lessard P, Ghaemmaghami S, Prusiner SB

Salivary prions in sheep and deer.

Prion

Tamgüney G, Richt JA, Hamir AN, Greenlee JJ, Miller MW, Wolfe LL, Sirochman TM, Young AJ, Glidden DV, Johnson NL, Giles K, DeArmond SJ, Prusiner SB

Prion uptake in the gut: identification of the first uptake and replication sites.

PLoS pathogens

Kujala P, Raymond CR, Romeijn M, Godsave SF, van Kasteren SI, Wille H, Prusiner SB, Mabbott NA, Peters PJ

Spontaneous generation of anchorless prions in transgenic mice.

Proceedings of the National Academy of Sciences of the United States of America

Stöhr J, Watts JC, Legname G, Oehler A, Lemus A, Nguyen HO, Sussman J, Wille H, DeArmond SJ, Prusiner SB, Giles K

Protease-resistant prions selectively decrease Shadoo protein.

PLoS pathogens

Watts JC, Stöhr J, Bhardwaj S, Wille H, Oehler A, Dearmond SJ, Giles K, Prusiner SB

De novo generation of prion strains.

Nature reviews. Microbiology

Colby DW, Prusiner SB

A data processing pipeline for mammalian proteome dynamics studies using stable isotope metabolic labeling.

Molecular & cellular proteomics : MCP

Guan S, Price JC, Prusiner SB, Ghaemmaghami S, Burlingame AL

Conformational transformation and selection of synthetic prion strains.

Journal of molecular biology

Ghaemmaghami S, Watts JC, Nguyen HO, Hayashi S, DeArmond SJ, Prusiner SB

Conserved properties of human and bovine prion strains on transmission to guinea pigs.

Laboratory investigation; a journal of technical methods and pathology

Safar JG, Giles K, Lessard P, Letessier F, Patel S, Serban A, Dearmond SJ, Prusiner SB

A survey of antiprion compounds reveals the prevalence of non-PrP molecular targets.

The Journal of biological chemistry

Poncet-Montange G, St Martin SJ, Bogatova OV, Prusiner SB, Shoichet BK, Ghaemmaghami S

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

Watts JC, Giles K, Grillo SK, Lemus A, DeArmond SJ, Prusiner SB

2-Aminothiazoles as therapeutic leads for prion diseases.

Journal of medicinal chemistry

Gallardo-Godoy A, Gever J, Fife KL, Silber BM, Prusiner SB, Renslo AR

Prions.

Cold Spring Harbor perspectives in biology

Colby DW, Prusiner SB

Analysis of proteome dynamics in the mouse brain.

Proceedings of the National Academy of Sciences of the United States of America

Price JC, Guan S, Burlingame A, Prusiner SB, Ghaemmaghami S

Human prion strain selection in transgenic mice.

Annals of neurology

Giles K, Glidden DV, Patel S, Korth C, Groth D, Lemus A, DeArmond SJ, Prusiner SB

Conformation-dependent high-affinity monoclonal antibodies to prion proteins.

Journal of immunology (Baltimore, Md. : 1950)

Stanker LH, Serban AV, Cleveland E, Hnasko R, Lemus A, Safar J, DeArmond SJ, Prusiner SB

Colloid formation by drugs in simulated intestinal fluid.

Journal of medicinal chemistry

Doak AK, Wille H, Prusiner SB, Shoichet BK

Generation of antisera to purified prions in lipid rafts.

Prion

Hnasko R, Serban AV, Carlson G, Prusiner SB, Stanker LH

Protease-sensitive synthetic prions.

PLoS pathogens

Colby DW, Wain R, Baskakov IV, Legname G, Palmer CG, Nguyen HO, Lemus A, Cohen FE, DeArmond SJ, Prusiner SB

Discovery of 2-aminothiazoles as potent antiprion compounds.

Journal of virology

Ghaemmaghami S, May BC, Renslo AR, Prusiner SB

Chemical induction of misfolded prion protein conformers in cell culture.

The Journal of biological chemistry

Ghaemmaghami S, Ullman J, Ahn M, St Martin S, Prusiner SB

Continuous quinacrine treatment results in the formation of drug-resistant prions.

PLoS pathogens

Ghaemmaghami S, Ahn M, Lessard P, Giles K, Legname G, DeArmond SJ, Prusiner SB

Design and construction of diverse mammalian prion strains.

Proceedings of the National Academy of Sciences of the United States of America

Colby DW, Giles K, Legname G, Wille H, Baskakov IV, DeArmond SJ, Prusiner SB

Natural and synthetic prion structure from X-ray fiber diffraction.

Proceedings of the National Academy of Sciences of the United States of America

Wille H, Bian W, McDonald M, Kendall A, Colby DW, Bloch L, Ollesch J, Borovinskiy AL, Cohen FE, Prusiner SB, Stubbs G

Asymptomatic deer excrete infectious prions in faeces.

Nature

Tamgüney G, Miller MW, Wolfe LL, Sirochman TM, Glidden DV, Palmer C, Lemus A, DeArmond SJ, Prusiner SB

Prion proteins with pathogenic and protective mutations show similar structure and dynamics.

Biochemistry

Bae SH, Legname G, Serban A, Prusiner SB, Wright PE, Dyson HJ

Measuring prions by bioluminescence imaging.

Proceedings of the National Academy of Sciences of the United States of America

Tamgüney G, Francis KP, Giles K, Lemus A, DeArmond SJ, Prusiner SB

Is Parkinson's disease a prion disorder?

Proceedings of the National Academy of Sciences of the United States of America

Olanow CW, Prusiner SB

Prion proteins in subpopulations of white blood cells from patients with sporadic Creutzfeldt-Jakob disease.

Laboratory investigation; a journal of technical methods and pathology

Choi EM, Geschwind MD, Deering C, Pomeroy K, Kuo A, Miller BL, Safar JG, Prusiner SB

Development of antibody fragments for immunotherapy of prion diseases.

The Biochemical journal

Campana V, Zentilin L, Mirabile I, Kranjc A, Casanova P, Giacca M, Prusiner SB, Legname G, Zurzolo C

Transmission of scrapie and sheep-passaged bovine spongiform encephalopathy prions to transgenic mice expressing elk prion protein.

The Journal of general virology

Tamgüney G, Miller MW, Giles K, Lemus A, Glidden DV, DeArmond SJ, Prusiner SB

Surface charge of polyoxometalates modulates polymerization of the scrapie prion protein.

Proceedings of the National Academy of Sciences of the United States of America

Wille H, Shanmugam M, Murugesu M, Ollesch J, Stubbs G, Long JR, Safar JG, Prusiner SB

Cryo-immunogold electron microscopy for prions: toward identification of a conversion site.

The Journal of neuroscience : the official journal of the Society for Neuroscience

Godsave SF, Wille H, Kujala P, Latawiec D, DeArmond SJ, Serban A, Prusiner SB, Peters PJ

Resistance of bovine spongiform encephalopathy (BSE) prions to inactivation.

PLoS pathogens

Giles K, Glidden DV, Beckwith R, Seoanes R, Peretz D, DeArmond SJ, Prusiner SB

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

Spilman P, Lessard P, Sattavat M, Bush C, Tousseyn T, Huang EJ, Giles K, Golde T, Das P, Fauq A, Prusiner SB, Dearmond SJ

Genes contributing to prion pathogenesis.

The Journal of general virology

Tamgüney G, Giles K, Glidden DV, Lessard P, Wille H, Tremblay P, Groth DF, Yehiely F, Korth C, Moore RC, Tatzelt J, Rubinstein E, Boucheix C, Yang X, Stanley P, Lisanti MP, Dwek RA, Rudd PM, Moskovitz J, Epstein CJ, Cruz TD, Kuziel WA, Maeda N, Sap J, Ashe KH, Carlson GA, Tesseur I, Wyss-Coray T, Mucke L, Weisgraber KH, Mahley RW, Cohen FE, Prusiner SB

Transmission and detection of prions in feces.

The Journal of infectious diseases

Safar JG, Lessard P, Tamgüney G, Freyman Y, Deering C, Letessier F, Dearmond SJ, Prusiner SB

Mechanisms of prion protein assembly into amyloid.

Proceedings of the National Academy of Sciences of the United States of America

Stöhr J, Weinmann N, Wille H, Kaimann T, Nagel-Steger L, Birkmann E, Panza G, Prusiner SB, Eigen M, Riesner D

Small-molecule aggregates inhibit amyloid polymerization.

Nature chemical biology

Feng BY, Toyama BH, Wille H, Colby DW, Collins SR, May BC, Prusiner SB, Weissman J, Shoichet BK

Prion detection by an amyloid seeding assay.

Proceedings of the National Academy of Sciences of the United States of America

Colby DW, Zhang Q, Wang S, Groth D, Legname G, Riesner D, Prusiner SB

Cell division modulates prion accumulation in cultured cells.

Proceedings of the National Academy of Sciences of the United States of America

Ghaemmaghami S, Phuan PW, Perkins B, Ullman J, May BC, Cohen FE, Prusiner SB

Influence of water, fat, and glycerol on the mechanism of thermal prion inactivation.

The Journal of biological chemistry

Müller H, Stitz L, Wille H, Prusiner SB, Riesner D

Electron crystallography of the scrapie prion protein complexed with heavy metals.

Archives of biochemistry and biophysics

Wille H, Govaerts C, Borovinskiy A, Latawiec D, Downing KH, Cohen FE, Prusiner SB

Polio and Nobel prizes: looking back 50 years.

Annals of neurology

Norrby E, Prusiner SB

Discriminating between cellular and misfolded prion protein by using affinity to 9-aminoacridine compounds.

The Journal of general virology

Phuan PW, Zorn JA, Safar J, Giles K, Prusiner SB, Cohen FE, May BC

Phosphorothioate oligonucleotides reduce PrP levels and prion infectivity in cultured cells.

Molecular medicine (Cambridge, Mass.)

Karpuj MV, Giles K, Gelibter-Niv S, Scott MR, Lingappa VR, Szoka FC, Peretz D, Denetclaw W, Prusiner SB

Transgenic mouse brains for the evaluation and quality control of BSE tests.

Biological chemistry

Philipp WJ, Groth D, Giles K, Vodrazka P, Schimmel H, Feyssaguet M, Toomik R, Schacher P, Osman AA, Lachmann I, Wear A, Arsac JN, Prusiner SB

Thioaptamer interactions with prion proteins: sequence-specific and non-specific binding sites.

Journal of molecular biology

King DJ, Safar JG, Legname G, Prusiner SB

Developmental expression of PrP in the post-implantation embryo.

Brain research

Tremblay P, Bouzamondo-Bernstein E, Heinrich C, Prusiner SB, DeArmond SJ

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

Legname G, Nguyen HO, Peretz D, Cohen FE, DeArmond SJ, Prusiner SB

Transmission of elk and deer prions to transgenic mice.

Journal of virology

Tamgüney G, Giles K, Bouzamondo-Bernstein E, Bosque PJ, Miller MW, Safar J, DeArmond SJ, Prusiner SB

Expression mapping of tetracycline-responsive prion protein promoter: digital atlasing for generating cell-specific disease models.

NeuroImage

Boy J, Leergaard TB, Schmidt T, Odeh F, Bichelmeier U, Nuber S, Holzmann C, Wree A, Prusiner SB, Bujard H, Riess O, Bjaalie JG

Structure-activity relationship study of 9-aminoacridine compounds in scrapie-infected neuroblastoma cells.

Bioorganic & medicinal chemistry letters

May BC, Witkop J, Sherrill J, Anderson MO, Madrid PB, Zorn JA, Prusiner SB, Cohen FE, Guy RK

Directed evolution of an anti-prion protein scFv fragment to an affinity of 1 pM and its structural interpretation.

Journal of molecular biology

Luginbühl B, Kanyo Z, Jones RM, Fletterick RJ, Prusiner SB, Cohen FE, Williamson RA, Burton DR, Plückthun A

Human prions and plasma lipoproteins.

Proceedings of the National Academy of Sciences of the United States of America

Safar JG, Wille H, Geschwind MD, Deering C, Latawiec D, Serban A, King DJ, Legname G, Weisgraber KH, Mahley RW, Miller BL, Dearmond SJ, Prusiner SB

Copper induces conformational changes in the N-terminal part of cell-surface PrPC.

Archives of virology

Leclerc E, Serban H, Prusiner SB, Burton DR, Williamson RA

Changes in neuropeptide expression in mice infected with prions.

Neurobiology of aging

Diez M, Groth D, DeArmond SJ, Prusiner SB, Hökfelt T

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

Huang Y, Okochi H, May BC, Legname G, Prusiner SB, Benet LZ, Guglielmo BJ, Lin ET

Prion infection of mouse neurospheres.

Proceedings of the National Academy of Sciences of the United States of America

Giri RK, Young R, Pitstick R, DeArmond SJ, Prusiner SB, Carlson GA

Discovering DNA encodes heredity and prions are infectious proteins.

Annual review of genetics

Prusiner SB, McCarty M

Inactivation of prions by acidic sodium dodecyl sulfate.

Journal of virology

Peretz D, Supattapone S, Giles K, Vergara J, Freyman Y, Lessard P, Safar JG, Glidden DV, McCulloch C, Nguyen HO, Scott M, Dearmond SJ, Prusiner SB

Recombinant prion protein induces rapid polarization and development of synapses in embryonic rat hippocampal neurons in vitro.

Journal of neurochemistry

Kanaani J, Prusiner SB, Diacovo J, Baekkeskov S, Legname G

Solid-state NMR structural studies of the fibril form of a mutant mouse prion peptide PrP89-143(P101L).

Solid state nuclear magnetic resonance

Lim KH, Nguyen TN, Damo SM, Mazur T, Ball HL, Prusiner SB, Pines A, Wemmer DE

Selective precipitation of prions by polyoxometalate complexes.

Journal of the American Chemical Society

Lee IS, Long JR, Prusiner SB, Safar JG

Prion clearance in bigenic mice.

The Journal of general virology

Safar JG, DeArmond SJ, Kociuba K, Deering C, Didorenko S, Bouzamondo-Bernstein E, Prusiner SB, Tremblay P

Search for a prion-specific nucleic acid.

Journal of virology

Safar JG, Kellings K, Serban A, Groth D, Cleaver JE, Prusiner SB, Riesner D

Assembly of natural and recombinant prion protein into fibrils.

Biological chemistry

Leffers KW, Wille H, Stöhr J, Junger E, Prusiner SB, Riesner D

Transmission barriers for bovine, ovine, and human prions in transgenic mice.

Journal of virology

Scott MR, Peretz D, Nguyen HO, Dearmond SJ, Prusiner SB

Diagnosis of human prion disease.

Proceedings of the National Academy of Sciences of the United States of America

Safar JG, Geschwind MD, Deering C, Didorenko S, Sattavat M, Sanchez H, Serban A, Vey M, Baron H, Giles K, Miller BL, Dearmond SJ, Prusiner SB

Strain-specified characteristics of mouse synthetic prions.

Proceedings of the National Academy of Sciences of the United States of America

Legname G, Nguyen HO, Baskakov IV, Cohen FE, Dearmond SJ, Prusiner SB

Notch-1 activation and dendritic atrophy in prion disease.

Proceedings of the National Academy of Sciences of the United States of America

Ishikura N, Clever JL, Bouzamondo-Bernstein E, Samayoa E, Prusiner SB, Huang EJ, DeArmond SJ

Oxidation of methionine residues in the prion protein by hydrogen peroxide.

Archives of biochemistry and biophysics

Requena JR, Dimitrova MN, Legname G, Teijeira S, Prusiner SB, Levine RL

Pharmacokinetics of quinacrine in the treatment of prion disease.

BMC infectious diseases

Yung L, Huang Y, Lessard P, Legname G, Lin ET, Baldwin M, Prusiner SB, Ryou C, Guglielmo BJ

Immunoglobulins in urine of hamsters with scrapie.

The Journal of biological chemistry

Serban A, Legname G, Hansen K, Kovaleva N, Prusiner SB

The neurodegeneration sequence in prion diseases: evidence from functional, morphological and ultrastructural studies of the GABAergic system.

Journal of neuropathology and experimental neurology

Bouzamondo-Bernstein E, Hopkins SD, Spilman P, Uyehara-Lock J, Deering C, Safar J, Prusiner SB, Ralston HJ, DeArmond SJ

Synthetic mammalian prions.

Science (New York, N.Y.)

Legname G, Baskakov IV, Nguyen HO, Riesner D, Cohen FE, DeArmond SJ, Prusiner SB

Detecting mad cow disease.

Scientific American

Prusiner SB

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

Govaerts C, Wille H, Prusiner SB, Cohen FE

Time-controlled transcardiac perfusion cross-linking for the study of protein interactions in complex tissues.

Nature biotechnology

Schmitt-Ulms G, Hansen K, Liu J, Cowdrey C, Yang J, DeArmond SJ, Cohen FE, Prusiner SB, Baldwin MA

Prions: so many fibers, so little infectivity.

Trends in biochemical sciences

May BC, Govaerts C, Prusiner SB, Cohen FE

The peculiar nature of unfolding of the human prion protein.

Protein science : a publication of the Protein Society

Baskakov IV, Legname G, Gryczynski Z, Prusiner SB

Mutant PrPSc conformers induced by a synthetic peptide and several prion strains.

Journal of virology

Tremblay P, Ball HL, Kaneko K, Groth D, Hegde RS, Cohen FE, DeArmond SJ, Prusiner SB, Safar JG

When sporadic disease is not sporadic: the potential for genetic etiology.

Archives of neurology

Goldman JS, Miller BL, Safar J, de Tourreil S, Martindale JL, Prusiner SB, Geschwind MD

Trafficking of prion proteins through a caveolae-mediated endosomal pathway.

The Journal of cell biology

Peters PJ, Mironov A, Peretz D, van Donselaar E, Leclerc E, Erpel S, DeArmond SJ, Burton DR, Williamson RA, Vey M, Prusiner SB

Cytosolic prion protein in neurons.

The Journal of neuroscience : the official journal of the Society for Neuroscience

Mironov A, Latawiec D, Wille H, Bouzamondo-Bernstein E, Legname G, Williamson RA, Burton D, DeArmond SJ, Prusiner SB, Peters PJ

Copper coordination in the full-length, recombinant prion protein.

Biochemistry

Burns CS, Aronoff-Spencer E, Legname G, Prusiner SB, Antholine WE, Gerfen GJ, Peisach J, Millhauser GL

Differential inhibition of prion propagation by enantiomers of quinacrine.

Laboratory investigation; a journal of technical methods and pathology

Ryou C, Legname G, Peretz D, Craig JC, Baldwin MA, Prusiner SB

Cooperative binding of dominant-negative prion protein to kringle domains.

Journal of molecular biology

Ryou C, Prusiner SB, Legname G

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

Korth C, Kaneko K, Groth D, Heye N, Telling G, Mastrianni J, Parchi P, Gambetti P, Will R, Ironside J, Heinrich C, Tremblay P, DeArmond SJ, Prusiner SB

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

May BC, Fafarman AT, Hong SB, Rogers M, Deady LW, Prusiner SB, Cohen FE

Conformation of PrP(C) on the cell surface as probed by antibodies.

Journal of molecular biology

Leclerc E, Peretz D, Ball H, Solforosi L, Legname G, Safar J, Serban A, Prusiner SB, Burton DR, Williamson RA

The PrP-like protein Doppel binds copper.

The Journal of biological chemistry

Qin K, Coomaraswamy J, Mastrangelo P, Yang Y, Lugowski S, Petromilli C, Prusiner SB, Fraser PE, Goldberg JM, Chakrabartty A, Westaway D

A change in the conformation of prions accompanies the emergence of a new prion strain.

Neuron

Peretz D, Williamson RA, Legname G, Matsunaga Y, Vergara J, Burton DR, DeArmond SJ, Prusiner SB, Scott MR

Pathway complexity of prion protein assembly into amyloid.

The Journal of biological chemistry

Baskakov IV, Legname G, Baldwin MA, Prusiner SB, Cohen FE

Prions in skeletal muscle.

Proceedings of the National Academy of Sciences of the United States of America

Bosque PJ, Ryou C, Telling G, Peretz D, Legname G, DeArmond SJ, Prusiner SB

Structural studies of the scrapie prion protein by electron crystallography.

Proceedings of the National Academy of Sciences of the United States of America

Wille H, Michelitsch MD, Guenebaut V, Supattapone S, Serban A, Cohen FE, Agard DA, Prusiner SB

Inherited prion disease caused by the V210I mutation: transmission to transgenic mice.

Neurology

Mastrianni JA, Capellari S, Telling GC, Han D, Bosque P, Prusiner SB, DeArmond SJ

Binding of neural cell adhesion molecules (N-CAMs) to the cellular prion protein.

Journal of molecular biology

Schmitt-Ulms G, Legname G, Baldwin MA, Ball HL, Bradon N, Bosque PJ, Crossin KL, Edelman GM, DeArmond SJ, Cohen FE, Prusiner SB

Doppel-induced cerebellar degeneration in transgenic mice.

Proceedings of the National Academy of Sciences of the United States of America

Moore RC, Mastrangelo P, Bouzamondo E, Heinrich C, Legname G, Prusiner SB, Hood L, Westaway D, DeArmond SJ, Tremblay P

Engineering the prion protein using chemical synthesis.

The journal of peptide research : official journal of the American Peptide Society

Ball HL, King DS, Cohen FE, Prusiner SB, Baldwin MA

Induction of distinct [URE3] yeast prion strains.

Molecular and cellular biology

Schlumpberger M, Prusiner SB, Herskowitz I

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

Laws DD, Bitter HM, Liu K, Ball HL, Kaneko K, Wille H, Cohen FE, Prusiner SB, Pines A, Wemmer DE

Shattuck lecture--neurodegenerative diseases and prions.

The New England journal of medicine

Prusiner SB

Folding of prion protein to its native alpha-helical conformation is under kinetic control.

The Journal of biological chemistry

Baskakov IV, Legname G, Prusiner SB, Cohen FE

Prion glycoprotein: structure, dynamics, and roles for the sugars.

Biochemistry

Rudd PM, Wormald MR, Wing DR, Prusiner SB, Dwek RA

Immobilized prion protein undergoes spontaneous rearrangement to a conformation having features in common with the infectious form.

The EMBO journal

Leclerc E, Peretz D, Ball H, Sakurai H, Legname G, Serban A, Prusiner SB, Burton DR, Williamson RA

A protease-resistant 61-residue prion peptide causes neurodegeneration in transgenic mice.

Molecular and cellular biology

Supattapone S, Bouzamondo E, Ball HL, Wille H, Nguyen HO, Cohen FE, DeArmond SJ, Prusiner SB, Scott M

Branched polyamines cure prion-infected neuroblastoma cells.

Journal of virology

Supattapone S, Wille H, Uyechi L, Safar J, Tremblay P, Szoka FC, Cohen FE, Prusiner SB, Scott MR

Conformational propagation with prion-like characteristics in a simple model of protein folding.

Protein science : a publication of the Protein Society

Harrison PM, Chan HS, Prusiner SB, Cohen FE

Strain-specified relative conformational stability of the scrapie prion protein.

Protein science : a publication of the Protein Society

Peretz D, Scott MR, Groth D, Williamson RA, Burton DR, Cohen FE, Prusiner SB

Local structural plasticity of the prion protein. Analysis of NMR relaxation dynamics.

Biochemistry

Viles JH, Donne D, Kroon G, Prusiner SB, Cohen FE, Dyson HJ, Wright PE

Two different neurodegenerative diseases caused by proteins with similar structures.

Proceedings of the National Academy of Sciences of the United States of America

Mo H, Moore RC, Cohen FE, Westaway D, Prusiner SB, Wright PE, Dyson HJ

Identification of two prion protein regions that modify scrapie incubation time.

Journal of virology

Supattapone S, Muramoto T, Legname G, Mehlhorn I, Cohen FE, DeArmond SJ, Prusiner SB, Scott MR

Affinity-tagged miniprion derivatives spontaneously adopt protease-resistant conformations.

Journal of virology

Supattapone S, Nguyen HO, Muramoto T, Cohen FE, DeArmond SJ, Prusiner SB, Scott M

Identification of the Cu2+ binding sites in the N-terminal domain of the prion protein by EPR and CD spectroscopy.

Biochemistry

Aronoff-Spencer E, Burns CS, Avdievich NI, Gerfen GJ, Peisach J, Antholine WE, Ball HL, Cohen FE, Prusiner SB, Millhauser GL

Quantitative trait loci affecting prion incubation time in mice.

Genomics

Stephenson DA, Chiotti K, Ebeling C, Groth D, DeArmond SJ, Prusiner SB, Carlson GA

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

Silverman GL, Qin K, Moore RC, Yang Y, Mastrangelo P, Tremblay P, Prusiner SB, Cohen FE, Westaway D

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

Inouye H, Bond J, Baldwin MA, Ball HL, Prusiner SB, Kirschner DA

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

Perrier V, Wallace AC, Kaneko K, Safar J, Prusiner SB, Cohen FE

Cultured cell sublines highly susceptible to prion infection.

Journal of virology

Bosque PJ, Prusiner SB

Dominant-negative inhibition of prion formation diminished by deletion mutagenesis of the prion protein.

Journal of virology

Zulianello L, Kaneko K, Scott M, Erpel S, Han D, Cohen FE, Prusiner SB

Self-assembly of recombinant prion protein of 106 residues.

Biochemistry

Baskakov IV, Aagaard C, Mehlhorn I, Wille H, Groth D, Baldwin MA, Prusiner SB, Cohen FE

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

Schlumpberger M, Wille H, Baldwin MA, Butler DA, Herskowitz I, Prusiner SB

Copper binding to octarepeat peptides of the prion protein monitored by mass spectrometry.

Protein science : a publication of the Protein Society

Whittal RM, Ball HL, Cohen FE, Burlingame AL, Prusiner SB, Baldwin MA

A synthetic peptide initiates Gerstmann-Sträussler-Scheinker (GSS) disease in transgenic mice.

Journal of molecular biology

Kaneko K, Ball HL, Wille H, Zhang H, Groth D, Torchia M, Tremblay P, Safar J, Prusiner SB, DeArmond SJ, Baldwin MA, Cohen FE

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

Scott MR, Will R, Ironside J, Nguyen HO, Tremblay P, DeArmond SJ, Prusiner SB

Transmissible and genetic prion diseases share a common pathway of neurodegeneration.

Nature

Hegde RS, Tremblay P, Groth D, DeArmond SJ, Prusiner SB, Lingappa VR

Elimination of prions by branched polyamines and implications for therapeutics.

Proceedings of the National Academy of Sciences of the United States of America

Supattapone S, Nguyen HO, Cohen FE, Prusiner SB, Scott MR

Kinetics of prion protein accumulation in the CNS of mice with experimental scrapie.

Journal of neuropathology and experimental neurology

Tatzelt J, Groth DF, Torchia M, Prusiner SB, DeArmond SJ

Glycosylation differences between the normal and pathogenic prion protein isoforms.

Proceedings of the National Academy of Sciences of the United States of America

Rudd PM, Endo T, Colominas C, Groth D, Wheeler SF, Harvey DJ, Wormald MR, Serban H, Prusiner SB, Kobata A, Dwek RA

Antibody binding defines a structure for an epitope that participates in the PrPC-->PrPSc conformational change.

Journal of molecular biology

Kanyo ZF, Pan KM, Williamson RA, Burton DR, Prusiner SB, Fletterick RJ, Cohen FE

Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel.

Journal of molecular biology

Moore RC, Lee IY, Silverman GL, Harrison PM, Strome R, Heinrich C, Karunaratne A, Pasternak SH, Chishti MA, Liang Y, Mastrangelo P, Wang K, Smit AF, Katamine S, Carlson GA, Cohen FE, Prusiner SB, Melton DW, Tremblay P, Hood LE, Westaway D

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

Nishida N, Tremblay P, Sugimoto T, Shigematsu K, Shirabe S, Petromilli C, Erpel SP, Nakaoke R, Atarashi R, Houtani T, Torchia M, Sakaguchi S, DeArmond SJ, Prusiner SB, Katamine S

Prion protein conformation in a patient with sporadic fatal insomnia.

The New England journal of medicine

Mastrianni JA, Nixon R, Layzer R, Telling GC, Han D, DeArmond SJ, Prusiner SB

Solution structure of Syrian hamster prion protein rPrP(90-231).

Biochemistry

Liu H, Farr-Jones S, Ulyanov NB, Llinas M, Marqusee S, Groth D, Cohen FE, Prusiner SB, James TL

Prion protein of 106 residues creates an artifical transmission barrier for prion replication in transgenic mice.

Cell

Supattapone S, Bosque P, Muramoto T, Wille H, Aagaard C, Peretz D, Nguyen HO, Heinrich C, Torchia M, Safar J, Cohen FE, DeArmond SJ, Prusiner SB, Scott M

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

Viles JH, Cohen FE, Prusiner SB, Goodin DB, Wright PE, Dyson HJ

Thermodynamics of model prions and its implications for the problem of prion protein folding.

Journal of molecular biology

Harrison PM, Chan HS, Prusiner SB, Cohen FE

Prions.

Proceedings of the National Academy of Sciences of the United States of America

Prusiner SB

Mapping the prion protein using recombinant antibodies.

Journal of virology

Williamson RA, Peretz D, Pinilla C, Ball H, Bastidas RB, Rozenshteyn R, Houghten RA, Prusiner SB, Burton DR

Rapid acquisition of beta-sheet structure in the prion protein prior to multimer formation.

Biological chemistry

Post K, Pitschke M, Schäfer O, Wille H, Appel TR, Kirsch D, Mehlhorn I, Serban H, Prusiner SB, Riesner D

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

Tremblay P, Meiner Z, Galou M, Heinrich C, Petromilli C, Lisse T, Cayetano J, Torchia M, Mobley W, Bujard H, DeArmond SJ, Prusiner SB

Complete genomic sequence and analysis of the prion protein gene region from three mammalian species.

Genome research

Lee IY, Westaway D, Smit AF, Wang K, Seto J, Chen L, Acharya C, Ankener M, Baskin D, Cooper C, Yao H, Prusiner SB, Hood LE

Eight prion strains have PrP(Sc) molecules with different conformations.

Nature medicine

Safar J, Wille H, Itri V, Groth D, Serban H, Torchia M, Cohen FE, Prusiner SB

The three-dimensional structure of prion protein: implications for prion disease.

Biochemical Society transactions

Baldwin MA, James TL, Cohen FE, Prusiner SB

The prion diseases.

Brain pathology (Zurich, Switzerland)

Prusiner SB

Prion protein selectively binds copper(II) ions.

Biochemistry

Stöckel J, Safar J, Wallace AC, Cohen FE, Prusiner SB

Neuropeptide Y: some viewpoints on a multifaceted peptide in the normal and diseased nervous system.

Brain research. Brain research reviews

Hökfelt T, Broberger C, Zhang X, Diez M, Kopp J, Xu Z, Landry M, Bao L, Schalling M, Koistinaho J, DeArmond SJ, Prusiner S, Gong J, Walsh JH

Prion protein biology.

Cell

Prusiner SB, Scott MR, DeArmond SJ, Cohen FE

A transmembrane form of the prion protein in neurodegenerative disease.

Science (New York, N.Y.)

Hegde RS, Mastrianni JA, Scott MR, DeFea KA, Tremblay P, Torchia M, DeArmond SJ, Prusiner SB, Lingappa VR

Changes in the localization of brain prion proteins during scrapie infection.

Neurology

DeArmond SJ, Mobley WC, DeMott DL, Barry RA, Beckstead JH, Prusiner SB

Molecular studies of prion diseases.

Progress in brain research

Safar J, Prusiner SB

Pathologic conformations of prion proteins.

Annual review of biochemistry

Cohen FE, Prusiner SB

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

Scott MR, Safar J, Telling G, Nguyen O, Groth D, Torchia M, Koehler R, Tremblay P, Walther D, Cohen FE, DeArmond SJ, Prusiner SB

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

Donne DG, Viles JH, Groth D, Mehlhorn I, James TL, Cohen FE, Prusiner SB, Wright PE, Dyson HJ

Prion protein expression in Chinese hamster ovary cells using a glutamine synthetase selection and amplification system.

Protein engineering

Blochberger TC, Cooper C, Peretz D, Tatzelt J, Griffith OH, Baldwin MA, Prusiner SB

Propagation of prion strains through specific conformers of the prion protein.

Journal of virology

Scott MR, Groth D, Tatzelt J, Torchia M, Tremblay P, DeArmond SJ, Prusiner SB

Selective neuronal targeting in prion disease.

Neuron

DeArmond SJ, Sánchez H, Yehiely F, Qiu Y, Ninchak-Casey A, Daggett V, Camerino AP, Cayetano J, Rogers M, Groth D, Torchia M, Tremblay P, Scott MR, Cohen FE, Prusiner SB

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

Diez M, Koistinaho J, Dearmond SJ, Groth D, Prusiner SB, Hökfelt T

A hypothalamic neuronal cell line persistently infected with scrapie prions exhibits apoptosis.

Journal of virology

Schätzl HM, Laszlo L, Holtzman DM, Tatzelt J, DeArmond SJ, Weiner RI, Mobley WC, Prusiner SB

A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform.

Journal of molecular biology

Peretz D, Williamson RA, Matsunaga Y, Serban H, Pinilla C, Bastidas RB, Rozenshteyn R, James TL, Houghten RA, Cohen FE, Prusiner SB, Burton DR

Prion diseases and the BSE crisis.

Science (New York, N.Y.)

Prusiner SB

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

Kaneko K, Zulianello L, Scott M, Cooper CM, Wallace AC, James TL, Cohen FE, Prusiner SB

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

James TL, Liu H, Ulyanov NB, Farr-Jones S, Zhang H, Donne DG, Kaneko K, Groth D, Mehlhorn I, Prusiner SB, Cohen FE

Molecular properties of complexes formed between the prion protein and synthetic peptides.

Journal of molecular biology

Kaneko K, Wille H, Mehlhorn I, Zhang H, Ball H, Cohen FE, Baldwin MA, Prusiner SB

Heritable disorder resembling neuronal storage disease in mice expressing prion protein with deletion of an alpha-helix.

Nature medicine

Muramoto T, DeArmond SJ, Scott M, Telling GC, Cohen FE, Prusiner SB

Kinetics and mechanism of amyloid formation by the prion protein H1 peptide as determined by time-dependent ESR.

Chemistry & biology

Lundberg KM, Stenland CJ, Cohen FE, Prusiner SB, Millhauser GL

N-terminally tagged prion protein supports prion propagation in transgenic mice.

Protein science : a publication of the Protein Society

Telling GC, Tremblay P, Torchia M, Dearmond SJ, Cohen FE, Prusiner SB

Physical studies of conformational plasticity in a recombinant prion protein.

Biochemistry

Zhang H, Stockel J, Mehlhorn I, Groth D, Baldwin MA, Prusiner SB, James TL, Cohen FE

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

Kaneko K, Vey M, Scott M, Pilkuhn S, Cohen FE, Prusiner SB

Failure to transmit disease from gray tremor mutant mice.

Journal of virology

Carlson GA, Banks S, Lund D, Reichert C, Groth D, Torchia M, Dearmond SJ, Prusiner SB

The prion folding problem.

Current opinion in structural biology

Harrison PM, Bamborough P, Daggett V, Prusiner SB, Cohen FE

Prion protein gene variation among primates.

Journal of molecular biology

Schätzl HM, Da Costa M, Taylor L, Cohen FE, Prusiner SB

Genetics of prions.

Annual review of genetics

Prusiner SB, Scott MR

Identification of candidate proteins binding to prion protein.

Neurobiology of disease

Yehiely F, Bamborough P, Da Costa M, Perry BJ, Thinakaran G, Cohen FE, Carlson GA, Prusiner SB

Recombinant scrapie-like prion protein of 106 amino acids is soluble.

Proceedings of the National Academy of Sciences of the United States of America

Muramoto T, Scott M, Cohen FE, Prusiner SB

Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity.

Science (New York, N.Y.)

Telling GC, Parchi P, DeArmond SJ, Cortelli P, Montagna P, Gabizon R, Mastrianni J, Lugaresi E, Gambetti P, Prusiner SB

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

Vey M, Pilkuhn S, Wille H, Nixon R, DeArmond SJ, Smart EJ, Anderson RG, Taraboulos A, Prusiner SB

Chemical chaperones interfere with the formation of scrapie prion protein.

The EMBO journal

Tatzelt J, Prusiner SB, Welch WJ

Molecular biology and pathogenesis of prion diseases.

Trends in biochemical sciences

Prusiner SB

Mutation of the prion protein gene at codon 208 in familial Creutzfeldt-Jakob disease.

Neurology

Mastrianni JA, Iannicola C, Myers RM, DeArmond S, Prusiner SB

Decreased receptor-mediated calcium response in prion-infected cells correlates with decreased membrane fluidity and IP3 release.

Neurology

Wong K, Qiu Y, Hyun W, Nixon R, VanCleff J, Sanchez-Salazar J, Prusiner SB, DeArmond SJ

Aberrant induction of neuropeptide Y mRNA in hippocampal CA3 pyramidal neurones in scrapie-infected mice.

Neuroreport

Diez M, Koistinaho J, DeArmond SJ, Camerino AP, Groth D, Caytano JC, Prusiner SB, Hökfelt T

Scrapie in mice deficient in apolipoprotein E or glial fibrillary acidic protein.

Neurology

Tatzelt J, Maeda N, Pekny M, Yang SL, Betsholtz C, Eliasson C, Cayetano J, Camerino AP, DeArmond SJ, Prusiner SB

Solid-state NMR studies of the prion protein H1 fragment.

Protein science : a publication of the Protein Society

Heller J, Kolbert AC, Larsen R, Ernst M, Bekker T, Baldwin M, Prusiner SB, Pines A, Wemmer DE

Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice.

Genes & development

Telling GC, Haga T, Torchia M, Tremblay P, DeArmond SJ, Prusiner SB

Circumventing tolerance to generate autologous monoclonal antibodies to the prion protein.

Proceedings of the National Academy of Sciences of the United States of America

Williamson RA, Peretz D, Smorodinsky N, Bastidas R, Serban H, Mehlhorn I, DeArmond SJ, Prusiner SB, Burton DR

Separation of scrapie prion infectivity from PrP amyloid polymers.

Journal of molecular biology

Wille H, Zhang GF, Baldwin MA, Cohen FE, Prusiner SB

High-level expression and characterization of a purified 142-residue polypeptide of the prion protein.

Biochemistry

Mehlhorn I, Groth D, Stöckel J, Moffat B, Reilly D, Yansura D, Willett WS, Baldwin M, Fletterick R, Cohen FE, Vandlen R, Henner D, Prusiner SB

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

Lledo PM, Tremblay P, DeArmond SJ, Prusiner SB, Nicoll RA

Disruption of prion rods generates 10-nm spherical particles having high alpha-helical content and lacking scrapie infectivity.

Journal of virology

Riesner D, Kellings K, Post K, Wille H, Serban H, Groth D, Baldwin MA, Prusiner SB

Abnormal plasma membrane properties and functions in prion-infected cell lines.

Cold Spring Harbor symposia on quantitative biology

DeArmond SJ, Qiu Y, Wong K, Nixon R, Hyun W, Prusiner SB, Mobley WC

Human prion diseases and neurodegeneration.

Current topics in microbiology and immunology

Prusiner SB

Insoluble wild-type and protease-resistant mutant prion protein in brains of patients with inherited prion disease.

Nature medicine

Gabizon R, Telling G, Meiner Z, Halimi M, Kahana I, Prusiner SB

Lipid composition in scrapie-infected mouse brain: prion infection increases the levels of dolichyl phosphate and ubiquinone.

Journal of neurochemistry

Guan Z, Söderberg M, Sindelar P, Prusiner SB, Kristensson K, Dallner G

Molecular biology and genetics of prion diseases.

Cold Spring Harbor symposia on quantitative biology

Prusiner SB

Prion protein amyloid: separation of scrapie infectivity from PrP polymers.

Ciba Foundation symposium

Wille H, Baldwin MA, Cohen FE, DeArmond SJ, Prusiner SB

Prion protein structure and scrapie replication: theoretical, spectroscopic, and genetic investigations.

Cold Spring Harbor symposia on quantitative biology

Bamborough P, Wille H, Telling GC, Yehiely F, Prusiner SB, Cohen FE

Scrapie prions: a three-dimensional model of an infectious fragment.

Folding & design

Huang Z, Prusiner SB, Cohen FE

Structures of prion proteins and conformational models for prion diseases.

Current topics in microbiology and immunology

Huang Z, Prusiner SB, Cohen FE

Transgenetics and gene targeting in studies of prion diseases.

Current topics in microbiology and immunology

Scott MR, Telling GC, Prusiner SB

Transgenetics and neuropathology of prion diseases.

Current topics in microbiology and immunology

DeArmond SJ, Prusiner SB

Transgenetics of prion diseases.

Current topics in microbiology and immunology

Prusiner SB

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

Kaneko K, Peretz D, Pan KM, Blochberger TC, Wille H, Gabizon R, Griffith OH, Cohen FE, Baldwin MA, Prusiner SB

Prion disease (PrP-A117V) presenting with ataxia instead of dementia.

Neurology

Mastrianni JA, Curtis MT, Oberholtzer JC, Da Costa MM, DeArmond S, Prusiner SB, Garbern JY

Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein.

Cell

Telling GC, Scott M, Mastrianni J, Gabizon R, Torchia M, Cohen FE, DeArmond SJ, Prusiner SB

X-ray diffraction of scrapie prion rods and PrP peptides.

Journal of molecular biology

Nguyen JT, Inouye H, Baldwin MA, Fletterick RJ, Cohen FE, Prusiner SB, Kirschner DA

Complete penetrance of Creutzfeldt-Jakob disease in Libyan Jews carrying the E200K mutation in the prion protein gene.

Molecular medicine (Cambridge, Mass.)

Spudich S, Mastrianni JA, Wrensch M, Gabizon R, Meiner Z, Kahana I, Rosenmann H, Kahana E, Prusiner SB

Prion protein isoforms, a convergence of biological and structural investigations.

The Journal of biological chemistry

Baldwin MA, Cohen FE, Prusiner SB

Conformational transitions in peptides containing two putative alpha-helices of the prion protein.

Journal of molecular biology

Zhang H, Kaneko K, Nguyen JT, Livshits TL, Baldwin MA, Cohen FE, James TL, Prusiner SB

Prion protein peptides induce alpha-helix to beta-sheet conformational transitions.

Biochemistry

Nguyen J, Baldwin MA, Cohen FE, Prusiner SB

Cholesterol depletion and modification of COOH-terminal targeting sequence of the prion protein inhibit formation of the scrapie isoform.

The Journal of cell biology

Taraboulos A, Scott M, Semenov A, Avrahami D, Laszlo L, Prusiner SB, Avraham D

Etiology and pathogenesis of prion diseases.

The American journal of pathology

DeArmond SJ, Prusiner SB

On safari with PrP: prion diseases of animals.

Trends in microbiology

Westaway D, Carlson GA, Prusiner SB

Scrapie prions selectively modify the stress response in neuroblastoma cells.

Proceedings of the National Academy of Sciences of the United States of America

Tatzelt J, Zuo J, Voellmy R, Scott M, Hartl U, Prusiner SB, Welch WJ

Prion protein gene variation among primates.

Journal of molecular biology

Schätzl HM, Da Costa M, Taylor L, Cohen FE, Prusiner SB

Prion protein transgenes and the neuropathology in prion diseases.

Brain pathology (Zurich, Switzerland)

DeArmond SJ, Prusiner SB

The prion diseases.

Scientific American

Prusiner SB

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

Telling GC, Scott M, Hsiao KK, Foster D, Yang SL, Torchia M, Sidle KC, Collinge J, DeArmond SJ, Prusiner SB

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

Hsiao KK, Groth D, Scott M, Yang SL, Serban H, Rapp D, Foster D, Torchia M, Dearmond SJ, Prusiner SB

Neurodegeneration in humans caused by prions.

The Western journal of medicine

Prusiner SB

Proposed three-dimensional structure for the cellular prion protein.

Proceedings of the National Academy of Sciences of the United States of America

Huang Z, Gabriel JM, Baldwin MA, Fletterick RJ, Prusiner SB, Cohen FE

Structure and polymorphism of the mouse prion protein gene.

Proceedings of the National Academy of Sciences of the United States of America

Westaway D, Cooper C, Turner S, Da Costa M, Carlson GA, Prusiner SB

Determinants of carboxyl-terminal domain translocation during prion protein biogenesis.

The Journal of biological chemistry

De Fea KA, Nakahara DH, Calayag MC, Yost CS, Mirels LF, Prusiner SB, Lingappa VR

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

Carlson GA, Ebeling C, Yang SL, Telling G, Torchia M, Groth D, Westaway D, DeArmond SJ, Prusiner SB

Inherited prion diseases.

Proceedings of the National Academy of Sciences of the United States of America

Prusiner SB

Structural clues to prion replication.

Science (New York, N.Y.)

Cohen FE, Pan KM, Huang Z, Baldwin M, Fletterick RJ, Prusiner SB

Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie.

Genes & development

Westaway D, Zuliani V, Cooper CM, Da Costa M, Neuman S, Jenny AL, Detwiler L, Prusiner SB

Human prion diseases.

Annals of neurology

Prusiner SB, Hsiao KK

Genetics of prion diseases and prion diversity in mice.

Philosophical transactions of the Royal Society of London. Series B, Biological sciences

Carlson GA, DeArmond SJ, Torchia M, Westaway D, Prusiner SB

Molecular biology and genetics of prion diseases.

Philosophical transactions of the Royal Society of London. Series B, Biological sciences

Prusiner SB

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

Gabizon R, Rosenman H, Meiner Z, Kahana I, Kahana E, Shugart Y, Ott J, Prusiner SB

Nucleic acids in prion preparations: unspecific background or essential component?

Philosophical transactions of the Royal Society of London. Series B, Biological sciences

Kellings K, Prusiner SB, Riesner D

The neuropathological phenotype in transgenic mice expressing different prion protein constructs.

Philosophical transactions of the Royal Society of London. Series B, Biological sciences

DeArmond SJ, Yang SL, Cayetano-Canlas J, Groth D, Prusiner SB

Biosynthesis of the prion proteins in scrapie-infected cells in culture.

Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicas

Taraboulos A, Scott M, Semenov A, Avrahami D, Prusiner SB

Prion protein (PrP) is not involved in the pathogenesis of spongiform encephalopathy in zitter rats.

Neuroscience letters

Gomi H, Ikeda T, Kunieda T, Itohara S, Prusiner SB, Yamanouchi K

Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins.

Cell

Westaway D, DeArmond SJ, Cayetano-Canlas J, Groth D, Foster D, Yang SL, Torchia M, Carlson GA, Prusiner SB

Biology and genetics of prion diseases.

Annual review of microbiology

Prusiner SB

Detecting prion protein gene mutations by denaturing gradient gel electrophoresis.

Human mutation

Fink JK, Peacock ML, Warren JT, Roses AD, Prusiner SB

Prion diseases and neurodegeneration.

Annual review of neuroscience

Prusiner SB, DeArmond SJ

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

Prusiner SB, Groth D, Serban A, Koehler R, Foster D, Torchia M, Burton D, Yang SL, DeArmond SJ

Genetic and infectious prion diseases.

Archives of neurology

Prusiner SB

Scrapie prions alter receptor-mediated calcium responses in cultured cells.

Neurology

Kristensson K, Feuerstein B, Taraboulos A, Hyun WC, Prusiner SB, DeArmond SJ

The neurochemistry of prion diseases.

Journal of neurochemistry

DeArmond SJ, Prusiner SB

Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease (CJD).

American journal of human genetics

Gabizon R, Rosenmann H, Meiner Z, Kahana I, Kahana E, Shugart Y, Ott J, Prusiner SB

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

DeArmond SJ, Yang SL, Lee A, Bowler R, Taraboulos A, Groth D, Prusiner SB

Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes.

Cell

Scott M, Groth D, Foster D, Torchia M, Yang SL, DeArmond SJ, Prusiner SB

Biology of prion diseases.

Journal of acquired immune deficiency syndromes

Prusiner SB

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

Rogers M, Yehiely F, Scott M, Prusiner SB

Attempts to restore scrapie prion infectivity after exposure to protein denaturants.

Proceedings of the National Academy of Sciences of the United States of America

Prusiner SB, Groth D, Serban A, Stahl N, Gabizon R

Delimiting the location of the scrapie prion incubation time gene on chromosome 2 of the mouse.

Genetics

Carlson GA, Ebeling C, Torchia M, Westaway D, Prusiner SB

Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing.

Biochemistry

Stahl N, Baldwin MA, Teplow DB, Hood L, Gibson BW, Burlingame AL, Prusiner SB

Immunologic and molecular biologic studies of prion proteins in bovine spongiform encephalopathy.

The Journal of infectious diseases

Prusiner SB, Füzi M, Scott M, Serban D, Serban H, Taraboulos A, Gabriel JM, Wells GA, Wilesmith JW, Bradley R

Transgenetic investigations of prion diseases of humans and animals.

Philosophical transactions of the Royal Society of London. Series B, Biological sciences

Prusiner SB

Analysis of nucleic acids in purified scrapie prion preparations.

Archives of virology. Supplementum

Kellings K, Meyer N, Mirenda C, Prusiner SB, Riesner D

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

Gasset M, Baldwin MA, Fletterick RJ, Prusiner SB

Prion encephalopathies of animals and humans.

Developments in biological standardization

Prusiner SB

Prions and nucleic acids: search for "residual" nucleic acids and screening for mutations in the PrP-gene.

Developments in biological standardization

Riesner D, Kellings K, Wiese U, Wulfert M, Mirenda C, Prusiner SB

Chemistry and biology of prions.

Biochemistry

Prusiner SB

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

Gasset M, Baldwin MA, Lloyd DH, Gabriel JM, Holtzman DM, Cohen F, Fletterick R, Prusiner SB

Attempts to convert the cellular prion protein into the scrapie isoform in cell-free systems.

Journal of virology

Raeber AJ, Borchelt DR, Scott M, Prusiner SB

Molecular cloning of a candidate chicken prion protein.

Proceedings of the National Academy of Sciences of the United States of America

Gabriel JM, Oesch B, Kretzschmar H, Scott M, Prusiner SB

Molecular cloning of a mink prion protein gene.

The Journal of general virology

Kretzschmar HA, Neumann M, Riethmüller G, Prusiner SB

Natural and experimental prion diseases of humans and animals.

Current opinion in neurobiology

Prusiner SB

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

Giaccone G, Verga L, Bugiani O, Frangione B, Serban D, Prusiner SB, Farlow MR, Ghetti B, Tagliavini F

Purification and properties of the cellular prion protein from Syrian hamster brain.

Protein science : a publication of the Protein Society

Pan KM, Stahl N, Prusiner SB

Evidence for synthesis of scrapie prion proteins in the endocytic pathway.

The Journal of biological chemistry

Borchelt DR, Taraboulos A, Prusiner SB

Regional mapping of prion proteins in brain.

Proceedings of the National Academy of Sciences of the United States of America

Taraboulos A, Jendroska K, Serban D, Yang SL, DeArmond SJ, Prusiner SB

Chimeric prion protein expression in cultured cells and transgenic mice.

Protein science : a publication of the Protein Society

Scott MR, Köhler R, Foster D, Prusiner SB

Synthesis and trafficking of prion proteins in cultured cells.

Molecular biology of the cell

Taraboulos A, Raeber AJ, Borchelt DR, Serban D, Prusiner SB

Presence of prion protein in peripheral tissues of Libyan Jews with Creutzfeldt-Jakob disease.

Neurology

Meiner Z, Halimi M, Polakiewicz RD, Prusiner SB, Gabizon R

Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters.

Genes & development

Hecker R, Taraboulos A, Scott M, Pan KM, Yang SL, Torchia M, Jendroska K, DeArmond SJ, Prusiner SB

Glycosylinositol phospholipid anchors of the scrapie and cellular prion proteins contain sialic acid.

Biochemistry

Stahl N, Baldwin MA, Hecker R, Pan KM, Burlingame AL, Prusiner SB

Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein.

Nature

Büeler H, Fischer M, Lang Y, Bluethmann H, Lipp HP, DeArmond SJ, Prusiner SB, Aguet M, Weissmann C

Further analysis of nucleic acids in purified scrapie prion preparations by improved return refocusing gel electrophoresis.

The Journal of general virology

Kellings K, Meyer N, Mirenda C, Prusiner SB, Riesner D

Linkage of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease to the prion protein gene.

Nature genetics

Dlouhy SR, Hsiao K, Farlow MR, Foroud T, Conneally PM, Johnson P, Prusiner SB, Hodes ME, Ghetti B

Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles.

Nature genetics

Hsiao K, Dlouhy SR, Farlow MR, Cass C, Da Costa M, Conneally PM, Hodes ME, Ghetti B, Prusiner SB

Prion protein mutation at codon 102 in an Italian family with Gerstmann-Sträussler-Scheinker syndrome.

Neurology

Kretzschmar HA, Kufer P, Riethmüller G, DeArmond S, Prusiner SB, Schiffer D

Ultrastructural localization of scrapie prion proteins in cytoplasmic vesicles of infected cultured cells.

Laboratory investigation; a journal of technical methods and pathology

McKinley MP, Taraboulos A, Kenaga L, Serban D, Stieber A, DeArmond SJ, Prusiner SB, Gonatas N

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)

Rogers M, Serban D, Gyuris T, Scott M, Torchia T, Prusiner SB

Prions and prion proteins.

FASEB journal : official publication of the Federation of American Societies for Experimental Biology

Stahl N, Prusiner SB

Electrospray mass spectrometry of the glycosylinositol phospholipid of the scrapie prion protein.

Cell biology international reports

Stahl N, Baldwin MA, Prusiner SB

Proteinase-resistant prion protein accumulation in Syrian hamster brain correlates with regional pathology and scrapie infectivity.

Neurology

Jendroska K, Heinzel FP, Torchia M, Stowring L, Kretzschmar HA, Kon A, Stern A, Prusiner SB, DeArmond SJ

Molecular biology and pathology of scrapie and the prion diseases of humans.

Brain pathology (Zurich, Switzerland)

Prusiner SB, DeArmond SJ

Molecular biology of prion diseases.

Science (New York, N.Y.)

Prusiner SB

A prion protein variant in a family with the telencephalic form of Gerstmann-Sträussler-Scheinker syndrome.

Neurology

Hsiao KK, Cass C, Schellenberg GD, Bird T, Devine-Gage E, Wisniewski H, Prusiner SB

Infectious and genetic manifestations of prion diseases.

Molecular plant-microbe interactions : MPMI

Prusiner SB, Westaway D

Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease.

The New England journal of medicine

Hsiao K, Meiner Z, Kahana E, Cass C, Kahana I, Avrahami D, Scarlato G, Abramsky O, Prusiner SB, Gabizon R

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

Tagliavini F, Prelli F, Ghiso J, Bugiani O, Serban D, Prusiner SB, Farlow MR, Ghetti B, Frangione B

Scrapie prion rod formation in vitro requires both detergent extraction and limited proteolysis.

Journal of virology

McKinley MP, Meyer RK, Kenaga L, Rahbar F, Cotter R, Serban A, Prusiner SB

Genetics of prion infections.

Trends in genetics : TIG

Carlson GA, Hsiao K, Oesch B, Westaway D, Prusiner SB

Acceleration of scrapie in trisomy 16----diploid aggregation chimeras.

Annals of neurology

Epstein CJ, Foster DB, DeArmond SJ, Prusiner SB

Molecular biology and transgenetics of prion diseases.

Critical reviews in biochemistry and molecular biology

Prusiner SB

Molecular genetics and transgenic model of Gertsmann-Sträussler-Scheinker disease.

Alzheimer disease and associated disorders

Hsiao K, Prusiner SB

Novel properties and biology of scrapie prions.

Current topics in microbiology and immunology

Prusiner SB

Prion biology and diseases.

Harvey lectures

Prusiner SB

Prion protein genes: evolutionary and functional aspects.

Current topics in microbiology and immunology

Oesch B, Westaway D, Prusiner SB

Search for a putative scrapie genome in purified prion fractions reveals a paucity of nucleic acids.

The Journal of general virology

Meyer N, Rosenbaum V, Schmidt B, Gilles K, Mirenda C, Groth D, Prusiner SB, Riesner D

Spontaneous neurodegeneration in transgenic mice with prion protein codon 101 proline----leucine substitution.

Annals of the New York Academy of Sciences

Hsiao K, Scott M, Foster D, DeArmond SJ, Groth D, Serban H, Prusiner SB

Ultrastructural studies of prions.

Current topics in microbiology and immunology

McKinley MP, Prusiner SB

Spontaneous neurodegeneration in transgenic mice with mutant prion protein.

Science (New York, N.Y.)

Hsiao KK, Scott M, Foster D, Groth DF, DeArmond SJ, Prusiner SB

Inherited human prion diseases.

Neurology

Hsiao K, Prusiner SB

Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication.

Cell

Prusiner SB, Scott M, Foster D, Pan KM, Groth D, Mirenda C, Torchia M, Yang SL, Serban D, Carlson GA

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

Taraboulos A, Rogers M, Borchelt DR, McKinley MP, Scott M, Serban D, Prusiner SB

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

Snow AD, Wight TN, Nochlin D, Koike Y, Kimata K, DeArmond SJ, Prusiner SB

Novel structure and genetics of prions causing neurodegeneration in humans and animals.

Biologicals : journal of the International Association of Biological Standardization

Prusiner SB

Link between scrapie and BSE?

Nature

Westaway D, Prusiner SB

Identification of cellular proteins binding to the scrapie prion protein.

Biochemistry

Oesch B, Teplow DB, Stahl N, Serban D, Hood LE, Prusiner SB

Scrapie prion proteins accumulate in the cytoplasm of persistently infected cultured cells.

The Journal of cell biology

Taraboulos A, Serban D, Prusiner SB

Unusual topogenic sequence directs prion protein biogenesis.

Science (New York, N.Y.)

Lopez CD, Yost CS, Prusiner SB, Myers RM, Lingappa VR

DNA length polymorphism 5' to the myelin basic protein gene is associated with multiple sclerosis.

Annals of neurology

Boylan KB, Takahashi N, Paty DW, Sadovnick AD, Diamond M, Hood LE, Prusiner SB

Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells.

The Journal of cell biology

Borchelt DR, Scott M, Taraboulos A, Stahl N, Prusiner SB

Three hamster species with different scrapie incubation times and neuropathological features encode distinct prion proteins.

Molecular and cellular biology

Lowenstein DH, Butler DA, Westaway D, McKinley MP, DeArmond SJ, Prusiner SB

Non-hydrophobic extracytoplasmic determinant of stop transfer in the prion protein.

Nature

Yost CS, Lopez CD, Prusiner SB, Myers RM, Lingappa VR

Prion liposomes.

The Biochemical journal

Gabizon R, Prusiner SB

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

McKinley MP, Longo FM, Valletta JS, Rahbar F, Neve RL, Prusiner SB, Mobley WC

Prion diseases of the central nervous system.

Monographs in pathology

Prusiner SB, DeArmond SJ

Rapid detection of Creutzfeldt-Jakob disease and scrapie prion proteins.

Neurology

Serban D, Taraboulos A, DeArmond SJ, Prusiner SB

Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques.

Cell

Scott M, Foster D, Mirenda C, Serban D, Coufal F, Wälchli M, Torchia M, Groth D, Carlson G, DeArmond SJ, Westaway D, Prusiner SB

Acceleration of scrapie in neonatal Syrian hamsters.

Neurology

McKinley MP, DeArmond SJ, Torchia M, Mobley WC, Prusiner SB

Primary structure of prion protein may modify scrapie isolate properties.

Proceedings of the National Academy of Sciences of the United States of America

Carlson GA, Westaway D, DeArmond SJ, Peterson-Torchia M, Prusiner SB

Unraveling prion diseases through molecular genetics.

Trends in neurosciences

Westaway D, Carlson GA, Prusiner SB

Creutzfeldt-Jakob disease and scrapie prions.

Alzheimer disease and associated disorders

Prusiner SB

Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome.

Nature

Hsiao K, Baker HF, Crow TJ, Poulter M, Owen F, Terwilliger JD, Westaway D, Ott J, Prusiner SB

Insertion in prion protein gene in familial Creutzfeldt-Jakob disease.

Lancet (London, England)

Owen F, Poulter M, Lofthouse R, Collinge J, Crow TJ, Risby D, Baker HF, Ridley RM, Hsiao K, Prusiner SB

Immunoaffinity purification and neutralization of scrapie prions.

Progress in clinical and biological research

Gabizon R, McKinley MP, Groth D, Westaway D, DeArmond SJ, Carlson GA, Prusiner SB

PrPSc in scrapie-infected hamster brain is spatially and temporally related to histopathology and infectivity titer.

Progress in clinical and biological research

De Armond SJ, Gonzales M, Mobley WC, Kon AA, Stern A, Prusiner H, Prusiner SB

Scrapie prions.

Annual review of microbiology

Prusiner SB

Sulfated glycosaminoglycans in amyloid plaques of prion diseases.

Acta neuropathologica

Snow AD, Kisilevsky R, Willmer J, Prusiner SB, DeArmond SJ

Genetics and polymorphism of the mouse prion gene complex: control of scrapie incubation time.

Molecular and cellular biology

Carlson GA, Goodman PA, Lovett M, Taylor BA, Marshall ST, Peterson-Torchia M, Westaway D, Prusiner SB

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

Mobley WC, Neve RL, Prusiner SB, McKinley MP

CNS amyloid proteins in neurodegenerative diseases.

Neurology

Roberts GW, Lofthouse R, Allsop D, Landon M, Kidd M, Prusiner SB, Crow TJ

Immunoaffinity purification and neutralization of scrapie prion infectivity.

Proceedings of the National Academy of Sciences of the United States of America

Gabizon R, McKinley MP, Groth D, Prusiner SB

Purification and properties of the cellular and scrapie hamster prion proteins.

European journal of biochemistry

Turk E, Teplow DB, Hood LE, Prusiner SB

Scrapie prion liposomes and rods exhibit target sizes of 55,000 Da.

Virology

Bellinger-Kawahara CG, Kempner E, Groth D, Gabizon R, Prusiner SB

Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins.

Journal of virology

Butler DA, Scott MR, Bockman JM, Borchelt DR, Taraboulos A, Hsiao KK, Kingsbury DT, Prusiner SB

Properties of scrapie prion protein liposomes.

The Journal of biological chemistry

Gabizon R, McKinley MP, Groth DF, Kenaga L, Prusiner SB

Prion protein gene expression in cultured cells.

Protein engineering

Scott MR, Butler DA, Bredesen DE, Wälchli M, Hsiao KK, Prusiner SB

Characterization of prion proteins with monospecific antisera to synthetic peptides.

Journal of immunology (Baltimore, Md. : 1950)

Barry RA, Vincent MT, Kent SB, Hood LE, Prusiner SB

Developmental regulation of prion protein mRNA in brain.

Ciba Foundation symposium

McKinley MP, Lingappa VR, Prusiner SB

Genetic control of prion incubation period in mice.

Ciba Foundation symposium

Carlson GA, Westaway D, Goodman PA, Peterson M, Marshall ST, Prusiner SB

Molecular structure, biology, and genetics of prions.

Advances in virus research

Prusiner SB

Properties of scrapie prion proteins in liposomes and amyloid rods.

Ciba Foundation symposium

Gabizon R, McKinley MP, Prusiner SB

Search for a scrapie-specific nucleic acid: a progress report.

Ciba Foundation symposium

Oesch B, Groth DF, Prusiner SB, Weissmann C

Prions and neurodegenerative diseases.

The New England journal of medicine

Prusiner SB

Evidence for a secretory form of the cellular prion protein.

Biochemistry

Hay B, Prusiner SB, Lingappa VR

Immuno-gold localization of prion filaments in scrapie-infected hamster brains.

Laboratory investigation; a journal of technical methods and pathology

Wiley CA, Burrola PG, Buchmeier MJ, Wooddell MK, Barry RA, Prusiner SB, Lampert PW

The burden of proof in linking AIDS to scrapie.

Nature

Braun MJ, Gonda MA, George DG, Bazan JF, Fletterick RJ, Prusiner SB

Distinct prion proteins in short and long scrapie incubation period mice.

Cell

Westaway D, Goodman PA, Mirenda CA, McKinley MP, Carlson GA, Prusiner SB

Scrapie prion protein contains a phosphatidylinositol glycolipid.

Cell

Stahl N, Borchelt DR, Hsiao K, Prusiner SB

Purified scrapie prions resist inactivation by procedures that hydrolyze, modify, or shear nucleic acids.

Virology

Bellinger-Kawahara C, Diener TO, McKinley MP, Groth DF, Smith DR, Prusiner SB

Changes in the localization of brain prion proteins during scrapie infection.

Neurology

DeArmond SJ, Mobley WC, DeMott DL, Barry RA, Beckstead JH, Prusiner SB

Formic acid pretreatment enhances immunostaining of cerebral and systemic amyloids.

Laboratory investigation; a journal of technical methods and pathology

Kitamoto T, Ogomori K, Tateishi J, Prusiner SB

Scrapie infection diminishes spines and increases varicosities of dendrites in hamsters: a quantitative Golgi analysis.

Journal of neuropathology and experimental neurology

Hogan RN, Baringer JR, Prusiner SB

Immunoblotting of Creutzfeldt-Jakob disease prion proteins: host species-specific epitopes.

Annals of neurology

Bockman JM, Prusiner SB, Tateishi J, Kingsbury DT

Purified prion proteins and scrapie infectivity copartition into liposomes.

Proceedings of the National Academy of Sciences of the United States of America

Gabizon R, McKinley MP, Prusiner SB

DNA length polymorphism located 5' to the human myelin basic protein gene.

American journal of human genetics

Boylan KB, Takahashi N, Diamond M, Hood LE, Prusiner SB

Developmental expression of prion protein gene in brain.

Developmental biology

McKinley MP, Hay B, Lingappa VR, Lieberburg I, Prusiner SB

Prions causing nervous system degeneration.

Laboratory investigation; a journal of technical methods and pathology

Prusiner SB, DeArmond SJ

AIDS virus and scrapie protein genes.

Nature

Fernando Bazan J, Fletterick RJ, Prusiner SB

Predicted secondary structure and membrane topology of the scrapie prion protein.

Protein engineering

Bazan JF, Fletterick RJ, McKinley MP, Prusiner SB

On the biology of prions.

Acta neuropathologica

Prusiner SB, Gabizon R, McKinley MP

Prions causing degenerative neurological diseases.

Annual review of medicine

Prusiner SB

Purified scrapie prions resist inactivation by UV irradiation.

Journal of virology

Bellinger-Kawahara C, Cleaver JE, Diener TO, Prusiner SB

Prions are novel infectious pathogens causing scrapie and Creutzfeldt-Jakob disease.

BioEssays : news and reviews in molecular, cellular and developmental biology

Prusiner SB

Prion-protein immunoreactivity in human transmissible dementias.

The New England journal of medicine

Roberts GW, Lofthouse R, Brown R, Crow TJ, Barry RA, Prusiner SB

Monoclonal antibodies to the cellular and scrapie prion proteins.

The Journal of infectious diseases

Barry RA, Prusiner SB

Linkage of prion protein and scrapie incubation time genes.

Cell

Carlson GA, Kingsbury DT, Goodman PA, Coleman S, Marshall ST, DeArmond S, Westaway D, Prusiner SB

Amyloid plaques in Creutzfeldt-Jakob disease stain with prion protein antibodies.

Annals of neurology

Kitamoto T, Tateishi J, Tashima T, Takeshita I, Barry RA, DeArmond SJ, Prusiner SB

Molecular cloning of a human prion protein cDNA.

DNA (Mary Ann Liebert, Inc.)

Kretzschmar HA, Stowring LE, Westaway D, Stubblebine WH, Prusiner SB, Dearmond SJ

Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene.

Cell

Basler K, Oesch B, Scott M, Westaway D, Wälchli M, Groth DF, McKinley MP, Prusiner SB, Weissmann C

Molecular characteristics of prion rods purified from scrapie-infected hamster brains.

The Journal of infectious diseases

McKinley MP, Braunfeld MB, Bellinger CG, Prusiner SB

Scrapie and cellular prion proteins share polypeptide epitopes.

The Journal of infectious diseases

Barry RA, Kent SB, McKinley MP, Meyer RK, DeArmond SJ, Hood LE, Prusiner SB

Separation and properties of cellular and scrapie prion proteins.

Proceedings of the National Academy of Sciences of the United States of America

Meyer RK, McKinley MP, Bowman KA, Braunfeld MB, Barry RA, Prusiner SB

Conservation of the cellular gene encoding the scrapie prion protein.

Nucleic acids research

Westaway D, Prusiner SB

H2 histamine receptors on the epithelial cells of choroid plexus.

Journal of neurochemistry

Crook RB, Farber MB, Prusiner SB

Biology and structure of scrapie prions.

International review of neurobiology

McKinley MP, Prusiner SB

Replication of scrapie prions in hamster eyes precedes retinal degeneration.

Ophthalmic research

Hogan RN, Bowman KA, Baringer JR, Prusiner SB

Scrapie prion proteins are synthesized in neurons.

The American journal of pathology

Kretzschmar HA, Prusiner SB, Stowring LE, DeArmond SJ

Transmission of scrapie in hamsters.

The Journal of infectious diseases

Prusiner SB, Cochran SP, Alpers MP

A microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions.

Neurology

Monteiro ML, Swanson RA, Coppeto JR, Cuneo RA, DeArmond SJ, Prusiner SB

Antibodies to the scrapie protein decorate prion rods.

Journal of immunology (Baltimore, Md. : 1950)

Barry RA, McKinley MP, Bendheim PE, Lewis GK, DeArmond SJ, Prusiner SB

Identification of prion amyloid filaments in scrapie-infected brain.

Cell

DeArmond SJ, McKinley MP, Barry RA, Braunfeld MB, McColloch JR, Prusiner SB

Scrapie PrP 27-30 is a sialoglycoprotein.

Journal of virology

Bolton DC, Meyer RK, Prusiner SB

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

Bendheim PE, Bockman JM, McKinley MP, Kingsbury DT, Prusiner SB

Creutzfeldt-Jakob disease prion proteins in human brains.

The New England journal of medicine

Bockman JM, Kingsbury DT, McKinley MP, Bendheim PE, Prusiner SB

Prions--infectious pathogens causing the spongiform encephalopathies.

CRC critical reviews in clinical neurobiology

Prusiner SB, Kingsbury DT

Scrapie and Creutzfeldt-Jakob disease prions.

Microbiological sciences

Prusiner SB, Barry RA, McKinley MP, Bellinger CG, Meyer RK, DeArmond SJ, Kingsbury DT

Scrapie prions, brain amyloid, and senile dementia.

Current topics in cellular regulation

Prusiner SB

Molecular characteristics of the major scrapie prion protein.

Biochemistry

Bolton DC, McKinley MP, Prusiner SB

Bitemporal hypometabolism in Creutzfeldt-Jakob disease measured by positron emission tomography with [18F]-2-fluorodeoxyglucose.

Journal of computer assisted tomography

Friedland RP, Prusiner SB, Jagust WJ, Budinger TF, Davis RL

Prions.

Scientific American

Prusiner SB

Antibodies to a scrapie prion protein.

Nature

Bendheim PE, Barry RA, DeArmond SJ, Stites DP, Prusiner SB

Purification and structural studies of a major scrapie prion protein.

Cell

Prusiner SB, Groth DF, Bolton DC, Kent SB, Hood LE

Some speculations about prions, amyloid, and Alzheimer's disease.

The New England journal of medicine

Prusiner SB

Prions: novel infectious pathogens.

Advances in virus research

Prusiner SB

Retinal degeneration in experimental Creutzfeldt-Jakob disease.

Laboratory investigation; a journal of technical methods and pathology

Hogan RN, Kingsbury DT, Baringer JR, Prusiner SB

Scrapie prions aggregate to form amyloid-like birefringent rods.

Cell

Prusiner SB, McKinley MP, Bowman KA, Bolton DC, Bendheim PE, Groth DF, Glenner GG

Replication of the scrapie agent in hamster brain precedes neuronal vacuolation.

Journal of neuropathology and experimental neurology

Baringer JR, Bowman KA, Prusiner SB

Genetic control of scrapie and Creutzfeldt-Jakob disease in mice.

Journal of immunology (Baltimore, Md. : 1950)

Kingsbury DT, Kasper KC, Stites DP, Watson JD, Hogan RN, Prusiner SB

Resistance of the scrapie agent to inactivation by psoralens.

Photochemistry and photobiology

McKinley MP, Masiarz FR, Isaacs ST, Hearst JE, Prusiner SB

Identification of a protein that purifies with the scrapie prion.

Science (New York, N.Y.)

Bolton DC, McKinley MP, Prusiner SB

Further purification and characterization of scrapie prions.

Biochemistry

Prusiner SB, Bolton DC, Groth DF, Bowman KA, Cochran SP, McKinley MP

Immunological studies of scrapie infection.

Journal of neuroimmunology

Kasper KC, Stites DP, Bowman KA, Panitch H, Prusiner SB

Viroids and prions.

Proceedings of the National Academy of Sciences of the United States of America

Diener TO, McKinley MP, Prusiner SB

Research on scrapie.

Lancet (London, England)

Prusiner SB

Kuru with incubation periods exceeding two decades.

Annals of neurology

Prusiner SB, Gajdusek C, Alpers MP

Novel proteinaceous infectious particles cause scrapie.

Science (New York, N.Y.)

Prusiner SB

Measurement of the scrapie agent using an incubation time interval assay.

Annals of neurology

Prusiner SB, Cochran SP, Groth DF, Downey DE, Bowman KA, Martinez HM

Reversible chemical modification of the scrapie agent.

Science (New York, N.Y.)

McKinley MP, Masiarz FR, Prusiner SB

Scrapie agent contains a hydrophobic protein.

Proceedings of the National Academy of Sciences of the United States of America

Prusiner SB, McKinley MP, Groth DF, Bowman KA, Mock NI, Cochran SP, Masiarz FR

Culture and characterization of epithelial cells from bovine choroid plexus.

Journal of neurochemistry

Crook RB, Kasagami H, Prusiner SB

Thiocyanate and hydroxyl ions inactivate the scrapie agent.

Proceedings of the National Academy of Sciences of the United States of America

Prusiner SB, Groth DF, McKinley MP, Cochran SP, Bowman KA, Kasper KC

Scrapie-associated particles in postsynaptic processes. Further ultrastructural studies.

Journal of neuropathology and experimental neurology

Baringer JR, Prusiner SB, Wong JS

Kinetics of D-glucose transport into renal membrane vesicles: measurements using a vacuum manifold apparatus.

Journal of biochemical and biophysical methods

Mamelok RD, Macrae DR, Hittelman K, Hoefer JP, Prusiner SB

Determination of scrapie agent titer from incubation period measurements in hamsters.

Advances in experimental medicine and biology

Prusiner SB, Cochran SP, Downey DE, Groth DF

Progressive retinal degeneration in scrapie-infected hamsters: a light and electron microscopic analysis.

Laboratory investigation; a journal of technical methods and pathology

Hogan RN, Baringer JR, Prusiner SB

Toward development of assays for scrapie-specific antibodies.

Advances in experimental medicine and biology

Kasper KC, Bowman K, Stites DP, Prusiner SB

Molecular properties, partial purification, and assay by incubation period measurements of the hamster scrapie agent.

Biochemistry

Prusiner SB, Groth DF, Cochran SP, Masiarz FR, McKinley MP, Martinez HM

Biohazards of investigations on the transmissible spongiform encephalopathies.

Reviews of infectious diseases

Chatigny MA, Prusiner SB

Experimental scrapie in the mouse: electrophoretic and sedimentation properties of the partially purified agent.

Journal of neurochemistry

Prusiner SB, Garfin DE, Cochran SP, McKinley MP, Groth DF, Hadlow WJ, Race RE, Eklund CM

Electrophoretic properties of the scrapie agent in agarose gels.

Proceedings of the National Academy of Sciences of the United States of America

Prusiner SB, Groth DF, Bildstein C, Masiarz FR, McKinley MP, Cochran SP

Slow viruses: molecular properties of the agents causing scrapie in mice and hamsters.

Progress in clinical and biological research

Prusiner SB, Cochran SP, Baringer JR, Groth D, Masiarz F, McKinley M, Bildstein C, Garfin D, Hadlow WJ, Race RE, Eklund CM

Evaluation of a class III biological safety cabinet for enclosure of an ultracentrifuge.

Applied and environmental microbiology

Chatigny MA, Dunn S, Ishimaru K, Eagleson JA, Prusiner SB

Partial purification and evidence for multiple molecular forms of the scrapie agent.

Biochemistry

Prusiner SB, Hadlow WJ, Garfin DE, Cochran SP, Baringer JR, Race RE, Eklund CM

Sedimentation characteristics of the scrapie agent from murine spleen and brain.

Biochemistry

Prusiner SB, Hadlow WJ, Eklund CM, Race RE, Cochran SP

Experimental scrapie in mice: ultrastructural observations.

Annals of neurology

Baringer JR, Prusiner SB

Mitogen stimulation of splenocytes from mice infected with scrapie agent.

The Journal of infectious diseases

Garfin DE, Stites DP, Perlman JD, Cochran SP, Prusiner SB

Preservation by freezing of glucose and alanine transport into kidney membrane vesicles.

Analytical biochemistry

Hittelman K, Mamelok RD, Prusiner SB

Suppression of polyclonal B cell activation in scrapie-infected C3H/HeJ mice.

Journal of immunology (Baltimore, Md. : 1950)

Garfin DE, Stites DP, Zitnik LA, Prusiner SB

Evidence for hydrophobic domains on the surface of the scrapie agent.

Transactions of the American Neurological Association

Prusiner SB, Garfin DE, Cochran SP, Baringer JR, Hadlow WJ, Eklund CM, Race RE

Sedimentation properties of the scrapie agent.

Proceedings of the National Academy of Sciences of the United States of America

Prusiner SB, Hadlow WJ, Eklund CM, Race RE

Carotidynia.

Neurology

Raskin NH, Prusiner S

Regulation of glutaminase levels in Escherichia coli.

Journal of bacteriology

Prusiner S

Stain for glutaminase activity.

Analytical biochemistry

Davis JN, Prusiner S

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

Prusiner S, Miller RE, Valentine RC

On the regulation of glutaminase in E. coli: metabolite control.

Biochemical and biophysical research communications

Prusiner S, Stadtman ER

Vacuum manifold for rapid assay of enzymes using radioactive tracers and ion exchange chromatography.

The Review of scientific instruments

Prusiner SB, Milner LS, Long CW, Myers ML

Metabolic control in isolated brown fat cells.

Lipids

Lindberg O, Prusiner SB, Cannon B, Ching TM, Eisenhardt RH

Control of metabolism in brown adipose tissue.

Lipids

Williamson JR, Prusiner S, Olson MS, Fukami M

[Energetic disturbance of cation transport as a cause of intracellular brain edema].

Acta neurochirurgica

Reulen HJ, Steude U, Brendel W, Hilber C, Prusiner S

Hypothermic protection against cerebral edema of ischemia.

Archives of neurology

Prusiner S, Wolfson SK

Oxidative metabolism in cells isolated from brown adipose tissue. 2. Catecholamine regulated respiratory control.

European journal of biochemistry

Prusiner SB, Cannon B, Ching TM, Lindberg O

The regulation of oxidative metabolism of isolated brown fat cells.

Biochemical and biophysical research communications

Prusiner SB, Eisenhardt RH, Rylander E, Lindberg O

Pyridine nucleotide changes during thermogenesis in brown fat tissue in vivo.

Archives of biochemistry and biophysics

Prusiner S, Williamson JR, Chance B, Paddle BM

Relationship of acidemia to cerebral edema.

Archives of surgery (Chicago, Ill. : 1960)

Prusiner SB, Moskovitz PA, Wolfson SK