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

Krejciova Z, Carlson GA, Giles K, Prusiner SB.

Acta Neuropathol Commun. 2019 May 20;7(1):81. doi: 10.1186/s40478-019-0703-9.


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

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.

Sci Transl Med. 2019 May 1;11(490). pii: eaat8462. doi: 10.1126/scitranslmed.aat8462.


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

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

Acta Neuropathol. 2019 Mar;137(3):437-454. doi: 10.1007/s00401-019-01959-4. Epub 2019 Jan 28.


A long-lived Aβ oligomer resistant to fibrillization.

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

Biopolymers. 2018 Aug;109(8):e23096. doi: 10.1002/bip.23096. Epub 2018 Jan 10.


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

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.

Proc Natl Acad Sci U S A. 2018 Jan 23;115(4):E782-E791. doi: 10.1073/pnas.1714966115. Epub 2018 Jan 8.


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

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

Proc Natl Acad Sci U S A. 2018 Jan 9;115(2):409-414. doi: 10.1073/pnas.1719369115. Epub 2017 Dec 26.


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

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

Proc Natl Acad Sci U S A. 2017 Dec 19;114(51):E11029-E11036. doi: 10.1073/pnas.1717193114. Epub 2017 Dec 4.


A novel vector for transgenesis in the rat CNS.

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

Acta Neuropathol Commun. 2017 Nov 21;5(1):84. doi: 10.1186/s40478-017-0484-y.


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

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

JAMA Neurol. 2017 Dec 1;74(12):1464-1472. doi: 10.1001/jamaneurol.2017.2822.


MSA prions exhibit remarkable stability and resistance to inactivation.

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

Acta Neuropathol. 2018 Jan;135(1):49-63. doi: 10.1007/s00401-017-1762-2. Epub 2017 Aug 28.


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

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.

Nat Chem. 2017 Sep;9(9):874-881. doi: 10.1038/nchem.2754. Epub 2017 Apr 3.


Bioassays and Inactivation of Prions.

Giles K, Woerman AL, Berry DB, Prusiner SB.

Cold Spring Harb Perspect Biol. 2017 Aug 1;9(8). pii: a023499. doi: 10.1101/cshperspect.a023499. Review.


α-Synuclein: Multiple System Atrophy Prions.

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

Cold Spring Harb Perspect Med. 2018 Jul 2;8(7). pii: a024588. doi: 10.1101/cshperspect.a024588. Review.


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

Watts JC, Prusiner SB.

Cold Spring Harb Perspect Med. 2018 May 1;8(5). pii: a023507. doi: 10.1101/cshperspect.a023507. Review.


Experimental Models of Inherited PrP Prion Diseases.

Watts JC, Prusiner SB.

Cold Spring Harb Perspect Med. 2017 Nov 1;7(11). pii: a027151. doi: 10.1101/cshperspect.a027151. Review.


Developing Therapeutics for PrP Prion Diseases.

Giles K, Olson SH, Prusiner SB.

Cold Spring Harb Perspect Med. 2017 Apr 3;7(4). pii: a023747. doi: 10.1101/cshperspect.a023747. Review.


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

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

Proc Natl Acad Sci U S A. 2016 Dec 13;113(50):E8187-E8196. Epub 2016 Nov 28.


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

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

J Virol. 2016 Oct 14;90(21):9558-9569. doi: 10.1128/JVI.01106-16. Print 2016 Nov 1.


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

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

J Am Chem Soc. 2016 Aug 10;138(31):9840-52. doi: 10.1021/jacs.6b03715. Epub 2016 Jul 28.


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

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

Proc Natl Acad Sci U S A. 2016 Jul 26;113(30):8514-9. doi: 10.1073/pnas.1607079113. Epub 2016 Jul 11.


Towards authentic transgenic mouse models of heritable PrP prion diseases.

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

Acta Neuropathol. 2016 Oct;132(4):593-610. doi: 10.1007/s00401-016-1585-6. Epub 2016 Jun 28.


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

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

J Pharmacol Exp Ther. 2016 Sep;358(3):537-47. doi: 10.1124/jpet.116.235556. Epub 2016 Jun 17.


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

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.

Proc Natl Acad Sci U S A. 2015 Sep 22;112(38):E5308-17. doi: 10.1073/pnas.1514475112. Epub 2015 Aug 31.


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

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

Biophys J. 2015 Aug 18;109(4):793-805. doi: 10.1016/j.bpj.2015.06.065.


Propagation of prions causing synucleinopathies in cultured cells.

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.

Proc Natl Acad Sci U S A. 2015 Sep 1;112(35):E4949-58. doi: 10.1073/pnas.1513426112. Epub 2015 Aug 18.


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

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.

J Pharmacol Exp Ther. 2015 Oct;355(1):2-12. doi: 10.1124/jpet.115.224659. Epub 2015 Jul 29.


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

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

J Infect Dis. 2015 Jul 15;212 Suppl 1:S17-25. doi: 10.1093/infdis/jiu656.


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

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

Ann Neurol. 2015 Oct;78(4):540-53. doi: 10.1002/ana.24463. Epub 2015 Aug 25.


Structural studies of truncated forms of the prion protein PrP.

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

Biophys J. 2015 Mar 24;108(6):1548-1554. doi: 10.1016/j.bpj.2015.01.008.


Mechanism of scrapie prion precipitation with phosphotungstate anions.

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

ACS Chem Biol. 2015 May 15;10(5):1269-77. doi: 10.1021/cb5006239. Epub 2015 Feb 19.


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

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

Proc Natl Acad Sci U S A. 2014 Jul 15;111(28):10323-8. doi: 10.1073/pnas.1408900111. Epub 2014 Jun 30.


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

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

Proc Natl Acad Sci U S A. 2014 Jul 15;111(28):10329-34. doi: 10.1073/pnas.1408968111. Epub 2014 Jun 30.


Mouse models for studying the formation and propagation of prions.

Watts JC, Prusiner SB.

J Biol Chem. 2014 Jul 18;289(29):19841-9. doi: 10.1074/jbc.R114.550707. Epub 2014 May 23. Review.


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

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

PLoS Pathog. 2014 Apr 3;10(4):e1003990. doi: 10.1371/journal.ppat.1003990. eCollection 2014 Apr.


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

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.

Bioorg Med Chem. 2014 Mar 15;22(6):1960-72. doi: 10.1016/j.bmc.2014.01.001. Epub 2014 Jan 9.


Biology and genetics of prions causing neurodegeneration.

Prusiner SB.

Annu Rev Genet. 2013;47:601-23. doi: 10.1146/annurev-genet-110711-155524. Review.


Transmission of multiple system atrophy prions to transgenic mice.

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

Proc Natl Acad Sci U S A. 2013 Nov 26;110(48):19555-60. doi: 10.1073/pnas.1318268110. Epub 2013 Nov 11.


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

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

Bioorg Med Chem. 2013 Dec 15;21(24):7999-8012. doi: 10.1016/j.bmc.2013.09.022. Epub 2013 Sep 18.


Drug resistance confounding prion therapeutics.

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

Proc Natl Acad Sci U S A. 2013 Oct 29;110(44):E4160-9. doi: 10.1073/pnas.1317164110. Epub 2013 Oct 15.


Quinacrine treatment trial for sporadic Creutzfeldt-Jakob disease.

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.

Neurology. 2013 Dec 3;81(23):2015-23. doi: 10.1212/WNL.0b013e3182a9f3b4. Epub 2013 Oct 11.


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

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

ACS Med Chem Lett. 2013 Jul 11;4(7):647-650.


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

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.

J Pharmacol Exp Ther. 2013 Nov;347(2):325-38. doi: 10.1124/jpet.113.205799. Epub 2013 Aug 21.


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

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

Curr Alzheimer Res. 2013 Sep;10(7):679-87.


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

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

ACS Med Chem Lett. 2013 Apr 11;4(4):397-401.


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

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.

ChemMedChem. 2013 May;8(5):847-57. doi: 10.1002/cmdc.201300007. Epub 2013 Mar 18.


Plasma membrane invaginations containing clusters of full-length PrPSc are an early form of prion-associated neuropathology in vivo.

Godsave SF, Wille H, Pierson J, Prusiner SB, Peters PJ.

Neurobiol Aging. 2013 Jun;34(6):1621-31. doi: 10.1016/j.neurobiolaging.2012.12.015.


Convergent replication of mouse synthetic prion strains.

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

Am J Pathol. 2013 Mar;182(3):866-74. doi: 10.1016/j.ajpath.2012.11.038.


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

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.

Pharm Res. 2013 Apr;30(4):932-50. doi: 10.1007/s11095-012-0912-4. Epub 2013 Feb 16.


Intracerebral Infusion of Antisense Oligonucleotides Into Prion-infected Mice.

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.

Mol Ther Nucleic Acids. 2012 Feb 7;1:e9. doi: 10.1038/mtna.2011.6.


Chimeric elk/mouse prion proteins in transgenic mice.

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

J Gen Virol. 2013 Feb;94(Pt 2):443-52. doi: 10.1099/vir.0.045989-0. Epub 2012 Oct 24.

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