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Early vs late age at onset frontotemporal dementia and frontotemporal lobar degeneration.

Seo SW, Thibodeau MP, Perry DC, Hua A, Sidhu M, Sible I, Vargas JNS, Gaus SE, Rabinovici GD, Rankin KD, Boxer AL, Kramer JH, Rosen HJ, Gorno-Tempini ML, Grinberg LT, Huang EJ, DeArmond SJ, Trojanowski JQ, Miller BL, Seeley WW.

Neurology. 2018 Mar 20;90(12):e1047-e1056. doi: 10.1212/WNL.0000000000005163. Epub 2018 Feb 16.


Autobiography Series: From Sleep-Wake Mechanisms to Prion Diseases.

DeArmond SJ.

J Neuropathol Exp Neurol. 2017 Jul 1;76(7):631-642. doi: 10.1093/jnen/nlx045. No abstract available.


Precipitous Deterioration of Motor Function, Cognition, and Behavior.

Fernández-Fournier M, Perry DC, Tartaglia MC, de May M, Boxer A, Coppola G, Christine CW, Huang EJ, Seeley WW, Miller BL, DeArmond SJ, Grinberg LT, Geschwind MD.

JAMA Neurol. 2017 May 1;74(5):591-596. doi: 10.1001/jamaneurol.2016.6159.


Brain Aggregates: An Effective In Vitro Cell Culture System Modeling Neurodegenerative Diseases.

Ahn M, Kalume F, Pitstick R, Oehler A, Carlson G, DeArmond SJ.

J Neuropathol Exp Neurol. 2016 Mar;75(3):256-62. doi: 10.1093/jnen/nlv025. Epub 2016 Feb 6. Erratum in: J Neuropathol Exp Neurol. 2016 May;75(5):479.


Selective Frontoinsular von Economo Neuron and Fork Cell Loss in Early Behavioral Variant Frontotemporal Dementia.

Kim EJ, Sidhu M, Gaus SE, Huang EJ, Hof PR, Miller BL, DeArmond SJ, Seeley WW.

Cereb Cortex. 2016 Apr;26(4):1843. doi: 10.1093/cercor/bhw012. Epub 2016 Jan 20. No abstract available.


Prion Disease Induces Alzheimer Disease-Like Neuropathologic Changes.

Tousseyn T, Bajsarowicz K, Sánchez H, Gheyara A, Oehler A, Geschwind M, DeArmond B, DeArmond SJ.

J Neuropathol Exp Neurol. 2015 Sep;74(9):873-88. doi: 10.1097/NEN.0000000000000228.


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.


Metabolic disorders with clinical and radiologic features of sporadic Creutzfeldt-Jakob disease.

Rosenbloom MH, Tartaglia MC, Forner SA, Wong KK, Kuo A, Johnson DY, Colacurcio V, Andrews BD, Miller BL, DeArmond SJ, Geschwind MD.

Neurol Clin Pract. 2015 Apr;5(2):108-115.


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.


White matter involvement in sporadic Creutzfeldt-Jakob disease.

Caverzasi E, Mandelli ML, DeArmond SJ, Hess CP, Vitali P, Papinutto N, Oehler A, Miller BL, Lobach IV, Bastianello S, Geschwind MD, Henry RG.

Brain. 2014 Dec;137(Pt 12):3339-54. doi: 10.1093/brain/awu298. Epub 2014 Nov 2.


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.


Convection-enhanced delivery of AAV2-PrPshRNA in prion-infected mice.

Ahn M, Bajsarowicz K, Oehler A, Lemus A, Bankiewicz K, DeArmond SJ.

PLoS One. 2014 May 27;9(5):e98496. doi: 10.1371/journal.pone.0098496. eCollection 2014.


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.


Application of quantitative DTI metrics in sporadic CJD.

Caverzasi E, Henry RG, Vitali P, Lobach IV, Kornak J, Bastianello S, Dearmond SJ, Miller BL, Rosen HJ, Mandelli ML, Geschwind MD.

Neuroimage Clin. 2014 Jan 31;4:426-35. doi: 10.1016/j.nicl.2014.01.011. eCollection 2014.


Genetic CJD with a novel E200G mutation in the prion protein gene and comparison with E200K mutation cases.

Kim MO, Cali I, Oehler A, Fong JC, Wong K, See T, Katz JS, Gambetti P, Bettcher BM, Dearmond SJ, Geschwind MD.

Acta Neuropathol Commun. 2013 Dec 12;1:80. doi: 10.1186/2051-5960-1-80.


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.


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.


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.


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.


Sporadic Jakob-Creutzfeldt disease presenting as primary progressive aphasia.

Johnson DY, Dunkelberger DL, Henry M, Haman A, Greicius MD, Wong K, DeArmond SJ, Miller BL, Gorno-Tempini ML, Geschwind MD.

JAMA Neurol. 2013 Feb;70(2):254-7. doi: 10.1001/2013.jamaneurol.139.


Levels of the Mahogunin Ring Finger 1 E3 ubiquitin ligase do not influence prion disease.

Silvius D, Pitstick R, Ahn M, Meishery D, Oehler A, Barsh GS, DeArmond SJ, Carlson GA, Gunn TM.

PLoS One. 2013;8(1):e55575. doi: 10.1371/journal.pone.0055575. Epub 2013 Jan 30.


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.


Differential diagnosis of Jakob-Creutzfeldt disease.

Paterson RW, Torres-Chae CC, Kuo AL, Ando T, Nguyen EA, Wong K, DeArmond SJ, Haman A, Garcia P, Johnson DY, Miller BL, Geschwind MD.

Arch Neurol. 2012 Dec;69(12):1578-82. doi: 10.1001/2013.jamaneurol.79.


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.


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

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

PLoS One. 2012;7(7):e39112. doi: 10.1371/journal.pone.0039112. Epub 2012 Jul 2.


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

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

Proc Natl Acad Sci U S A. 2012 Jul 3;109(27):11025-30. doi: 10.1073/pnas.1206555109. Epub 2012 Jun 18.


Clinical overlap between Jakob-Creutzfeldt disease and Lewy body disease.

Tartaglia MC, Johnson DY, Thai JN, Cattaruzza T, Wong K, Garcia P, Dearmond SJ, Miller BL, Geschwind MD.

Can J Neurol Sci. 2012 May;39(3):304-10.


A brain aggregate model gives new insights into the pathobiology and treatment of prion diseases.

Bajsarowicz K, Ahn M, Ackerman L, Dearmond BN, Carlson G, DeArmond SJ.

J Neuropathol Exp Neurol. 2012 May;71(5):449-66. doi: 10.1097/NEN.0b013e3182544680.


Salivary prions in sheep and deer.

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. 2012 Jan-Mar;6(1):52-61. doi: 10.4161/pri.6.1.16984.


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

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

J Virol. 2012 Jun;86(11):6033-41. doi: 10.1128/JVI.07027-11. Epub 2012 Mar 21.


Patterns of striatal degeneration in frontotemporal dementia.

Halabi C, Halabi A, Dean DL, Wang PN, Boxer AL, Trojanowski JQ, Dearmond SJ, Miller BL, Kramer JH, Seeley WW.

Alzheimer Dis Assoc Disord. 2013 Jan-Mar;27(1):74-83. doi: 10.1097/WAD.0b013e31824a7df4.


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

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

Proc Natl Acad Sci U S A. 2012 Feb 28;109(9):3498-503. doi: 10.1073/pnas.1121556109. Epub 2012 Feb 13.


Protease-resistant prions selectively decrease Shadoo protein.

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

PLoS Pathog. 2011 Nov;7(11):e1002382. doi: 10.1371/journal.ppat.1002382. Epub 2011 Nov 17.


Spontaneous generation of anchorless prions in transgenic mice.

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

Proc Natl Acad Sci U S A. 2011 Dec 27;108(52):21223-8. doi: 10.1073/pnas.1117827108. Epub 2011 Dec 12.


Amyloid vs FDG-PET in the differential diagnosis of AD and FTLD.

Rabinovici GD, Rosen HJ, Alkalay A, Kornak J, Furst AJ, Agarwal N, Mormino EC, O'Neil JP, Janabi M, Karydas A, Growdon ME, Jang JY, Huang EJ, Dearmond SJ, Trojanowski JQ, Grinberg LT, Gorno-Tempini ML, Seeley WW, Miller BL, Jagust WJ.

Neurology. 2011 Dec 6;77(23):2034-42. doi: 10.1212/WNL.0b013e31823b9c5e. Epub 2011 Nov 30.


Down-regulation of Shadoo in prion infections traces a pre-clinical event inversely related to PrP(Sc) accumulation.

Westaway D, Genovesi S, Daude N, Brown R, Lau A, Lee I, Mays CE, Coomaraswamy J, Canine B, Pitstick R, Herbst A, Yang J, Ko KW, Schmitt-Ulms G, Dearmond SJ, McKenzie D, Hood L, Carlson GA.

PLoS Pathog. 2011 Nov;7(11):e1002391. doi: 10.1371/journal.ppat.1002391. Epub 2011 Nov 17.


Safety and tolerability of magnetic resonance imaging-guided convection-enhanced delivery of AAV2-hAADC with a novel delivery platform in nonhuman primate striatum.

San Sebastian W, Richardson RM, Kells AP, Lamarre C, Bringas J, Pivirotto P, Salegio EA, Dearmond SJ, Forsayeth J, Bankiewicz KS.

Hum Gene Ther. 2012 Feb;23(2):210-7. doi: 10.1089/hum.2011.162. Epub 2012 Jan 26.


Behavioral variant frontotemporal dementia with corticobasal degeneration pathology: phenotypic comparison to bvFTD with Pick's disease.

Rankin KP, Mayo MC, Seeley WW, Lee S, Rabinovici G, Gorno-Tempini ML, Boxer AL, Weiner MW, Trojanowski JQ, DeArmond SJ, Miller BL.

J Mol Neurosci. 2011 Nov;45(3):594-608. doi: 10.1007/s12031-011-9615-2. Epub 2011 Sep 1.


Conformational transformation and selection of synthetic prion strains.

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

J Mol Biol. 2011 Oct 28;413(3):527-42. doi: 10.1016/j.jmb.2011.07.021. Epub 2011 Aug 4.


Clinicopathological correlations in corticobasal degeneration.

Lee SE, Rabinovici GD, Mayo MC, Wilson SM, Seeley WW, DeArmond SJ, Huang EJ, Trojanowski JQ, Growdon ME, Jang JY, Sidhu M, See TM, Karydas AM, Gorno-Tempini ML, Boxer AL, Weiner MW, Geschwind MD, Rankin KP, Miller BL.

Ann Neurol. 2011 Aug;70(2):327-40. doi: 10.1002/ana.22424.


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

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

Lab Invest. 2011 Sep;91(9):1326-36. doi: 10.1038/labinvest.2011.89. Epub 2011 Jul 4.


Selective frontoinsular von Economo neuron and fork cell loss in early behavioral variant frontotemporal dementia.

Kim EJ, Sidhu M, Gaus SE, Huang EJ, Hof PR, Miller BL, DeArmond SJ, Seeley WW.

Cereb Cortex. 2012 Feb;22(2):251-9. doi: 10.1093/cercor/bhr004. Epub 2011 Jun 8. Erratum in: Cereb Cortex. 2016 Apr;26(4):1843.


Bioluminescence imaging of Abeta deposition in bigenic mouse models of Alzheimer's disease.

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

Proc Natl Acad Sci U S A. 2011 Feb 8;108(6):2528-33. doi: 10.1073/pnas.1019034108. Epub 2011 Jan 24. Erratum in: Proc Natl Acad Sci U S A. 2015 Mar 3;112(9):E1050. Proc Natl Acad Sci U S A. 2014 Mar 11;111(10):3895.


TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia.

Rohrer JD, Geser F, Zhou J, Gennatas ED, Sidhu M, Trojanowski JQ, Dearmond SJ, Miller BL, Seeley WW.

Neurology. 2010 Dec 14;75(24):2204-11. doi: 10.1212/WNL.0b013e318202038c.


Pathologic evidence that the T188R mutation in PRNP is associated with prion disease.

Tartaglia MC, Thai JN, See T, Kuo A, Harbaugh R, Raudabaugh B, Cali I, Sattavat M, Sanchez H, DeArmond SJ, Geschwind MD.

J Neuropathol Exp Neurol. 2010 Dec;69(12):1220-7. doi: 10.1097/NEN.0b013e3181ffc39c.


Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein.

Zou WQ, Puoti G, Xiao X, Yuan J, Qing L, Cali I, Shimoji M, Langeveld JP, Castellani R, Notari S, Crain B, Schmidt RE, Geschwind M, Dearmond SJ, Cairns NJ, Dickson D, Honig L, Torres JM, Mastrianni J, Capellari S, Giaccone G, Belay ED, Schonberger LB, Cohen M, Perry G, Kong Q, Parchi P, Tagliavini F, Gambetti P.

Ann Neurol. 2010 Aug;68(2):162-72. doi: 10.1002/ana.22094.


Human prion strain selection in transgenic mice.

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

Ann Neurol. 2010 Aug;68(2):151-61. doi: 10.1002/ana.22104.

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