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Items: 1 to 50 of 75

1.

Sporadic Fatal Insomnia in Europe: Phenotypic Features and Diagnostic Challenges.

Abu-Rumeileh S, Redaelli V, Baiardi S, Mackenzie G, Windl O, Ritchie DL, Didato G, Hernandez-Vara J, Rossi M, Capellari S, Imperiale D, Rizzone MG, Belotti A, Sorbi S, Rozemuller AJM, Cortelli P, Gelpi E, Will RG, Zerr I, Giaccone G, Parchi P.

Ann Neurol. 2018 Sep;84(3):347-360. doi: 10.1002/ana.25300. Epub 2018 Aug 31.

PMID:
30048013
2.

Substitutions of PrP N-terminal histidine residues modulate scrapie disease pathogenesis and incubation time in transgenic mice.

Eigenbrod S, Frick P, Bertsch U, Mitteregger-Kretzschmar G, Mielke J, Maringer M, Piening N, Hepp A, Daude N, Windl O, Levin J, Giese A, Sakthivelu V, Tatzelt J, Kretzschmar H, Westaway D.

PLoS One. 2017 Dec 8;12(12):e0188989. doi: 10.1371/journal.pone.0188989. eCollection 2017.

3.

Effect of Polymorphisms at Codon 146 of the Goat PRNP Gene on Susceptibility to Challenge with Classical Scrapie by Different Routes.

Papasavva-Stylianou P, Simmons MM, Ortiz-Pelaez A, Windl O, Spiropoulos J, Georgiadou S.

J Virol. 2017 Oct 27;91(22). pii: e01142-17. doi: 10.1128/JVI.01142-17. Print 2017 Nov 15.

4.

Goats with aspartic acid or serine at codon 146 of the PRNP gene remain scrapie-negative after lifetime exposure in affected herds in Cyprus.

Georgiadou S, Ortiz-Pelaez A, Simmons MM, Windl O, Dawson M, Neocleous P, Papasavva-Stylianou P.

Epidemiol Infect. 2017 Jan;145(2):326-328. Epub 2016 Oct 18.

PMID:
27751198
5.

Prion protein genotype survey confirms low frequency of scrapie-resistant K222 allele in British goat herds.

Goldmann W, Marier E, Stewart P, Konold T, Street S, Langeveld J, Windl O, Ortiz-Pelaez A.

Vet Rec. 2016 Feb 13;178(7):168. doi: 10.1136/vr.103521. Epub 2016 Jan 11.

6.

Generation of a persistently infected MDBK cell line with natural bovine spongiform encephalopathy (BSE).

Tark D, Kim H, Neale MH, Kim M, Sohn H, Lee Y, Cho I, Joo Y, Windl O.

PLoS One. 2015 Feb 3;10(2):e0115939. doi: 10.1371/journal.pone.0115939. eCollection 2015.

7.

Allelic variants at codon 146 in the PRNP gene show significant differences in the risk for natural scrapie in Cypriot goats.

Ortiz-Pelaez A, Georgiadou S, Simmons MM, Windl O, Dawson M, Arnold ME, Neocleous P, Papasavva-Stylianou P.

Epidemiol Infect. 2015 Apr;143(6):1304-10. doi: 10.1017/S0950268814002064. Epub 2014 Aug 20.

PMID:
25140573
8.

Enzymatic formulation capable of degrading scrapie prion under mild digestion conditions.

Okoroma EA, Purchase D, Garelick H, Morris R, Neale MH, Windl O, Abiola OO.

PLoS One. 2013 Jul 16;8(7):e68099. doi: 10.1371/journal.pone.0068099. Print 2013.

9.

Overexpression of chimaeric murine/ovine PrP (A136H154Q171) in transgenic mice facilitates transmission and differentiation of ruminant prions.

Griffiths PC, Plater JM, Chave A, Jayasena D, Tout AC, Rice PB, Vickery CM, Spiropoulos J, Stack MJ, Windl O.

J Gen Virol. 2013 Nov;94(Pt 11):2577-86. doi: 10.1099/vir.0.051581-0. Epub 2013 Jun 12.

PMID:
23761404
10.

Spongiform encephalopathy in siblings with no evidence of protease-resistant prion protein or a mutation in the prion protein gene.

Varges D, Schulz-Schaeffer WJ, Wemheuer WM, Damman I, Schmitz M, Cramm M, Kallenberg K, Shirneshan K, Elkenani M, Markwort S, Faist M, Kohlhase J, Windl O, Zerr I.

J Neurol. 2013 Jul;260(7):1871-9. doi: 10.1007/s00415-013-6897-z. Epub 2013 Apr 2.

PMID:
23546304
11.

Four BSE cases with an L-BSE molecular profile in cattle from Great Britain.

Stack MJ, Chaplin MJ, Davis LA, Everitt S, Simmons MM, Windl O, Hope J, Burke P.

Vet Rec. 2013 Jan 19;172(3):70. doi: 10.1136/vr.101158. Epub 2012 Dec 18.

PMID:
23249774
12.

Animal prion diseases.

Windl O, Dawson M.

Subcell Biochem. 2012;65:497-516. doi: 10.1007/978-94-007-5416-4_18. Review.

PMID:
23225014
13.

In vitro amplification of ovine prions from scrapie-infected sheep from Great Britain reveals distinct patterns of propagation.

Thorne L, Holder T, Ramsay A, Edwards J, Taema MM, Windl O, Maddison BC, Gough KC, Terry LA.

BMC Vet Res. 2012 Nov 15;8:223. doi: 10.1186/1746-6148-8-223.

14.

Caprine prion gene polymorphisms are associated with decreased incidence of classical scrapie in goat herds in the United Kingdom.

Goldmann W, Ryan K, Stewart P, Parnham D, Xicohtencatl R, Fernandez N, Saunders G, Windl O, González L, Bossers A, Foster J.

Vet Res. 2011 Oct 31;42:110. doi: 10.1186/1297-9716-42-110.

15.

Transcriptional analysis implicates endoplasmic reticulum stress in bovine spongiform encephalopathy.

Tang Y, Xiang W, Terry L, Kretzschmar HA, Windl O.

PLoS One. 2010 Dec 3;5(12):e14207. doi: 10.1371/journal.pone.0014207.

16.

Proteomic consequences of expression and pathological conversion of the prion protein in inducible neuroblastoma N2a cells.

Provansal M, Roche S, Pastore M, Casanova D, Belondrade M, Alais S, Leblanc P, Windl O, Lehmann S.

Prion. 2010 Oct-Dec;4(4):292-301. doi: 10.4161/pri.4.4.13435. Epub 2010 Oct 27.

17.

PrP gene polymorphisms in Cyprus goats and their association with resistance or susceptibility to natural scrapie.

Papasavva-Stylianou P, Windl O, Saunders G, Mavrikiou P, Toumazos P, Kakoyiannis C.

Vet J. 2011 Feb;187(2):245-50. doi: 10.1016/j.tvjl.2009.10.015. Epub 2010 Jan 25.

PMID:
20093056
18.

Infection of cell lines with experimental and natural ovine scrapie agents.

Neale MH, Mountjoy SJ, Edwards JC, Vilette D, Laude H, Windl O, Saunders GC.

J Virol. 2010 Mar;84(5):2444-52. doi: 10.1128/JVI.01855-09. Epub 2009 Dec 23.

19.

High prevalence of scrapie in a dairy goat herd: tissue distribution of disease-associated PrP and effect of PRNP genotype and age.

González L, Martin S, Sisó S, Konold T, Ortiz-Peláez A, Phelan L, Goldmann W, Stewart P, Saunders G, Windl O, Jeffrey M, Hawkins SA, Dawson M, Hope J.

Vet Res. 2009 Nov-Dec;40(6):65. doi: 10.1051/vetres/2009048. Epub 2009 Aug 18.

20.

Protective effect of the T112 PrP variant in sheep challenged with bovine spongiform encephalopathy.

Saunders GC, Lantier I, Cawthraw S, Berthon P, Moore SJ, Arnold ME, Windl O, Simmons MM, Andréoletti O, Bellworthy S, Lantier F.

J Gen Virol. 2009 Oct;90(Pt 10):2569-74. doi: 10.1099/vir.0.012724-0. Epub 2009 Jul 8.

PMID:
19587133
21.

Transcriptional changes in the brains of cattle orally infected with the bovine spongiform encephalopathy agent precede detection of infectivity.

Tang Y, Xiang W, Hawkins SA, Kretzschmar HA, Windl O.

J Virol. 2009 Sep;83(18):9464-73. doi: 10.1128/JVI.00352-09. Epub 2009 Jul 8.

22.

Real-time PCR detection and identification of prohibited mammalian and avian material in animal feeds.

Cawthraw S, Saunders GC, Martin TC, Sawyer J, Windl O, Reaney SD.

J Food Prot. 2009 May;72(5):1055-62.

PMID:
19517734
23.

Ovine PRNP untranslated region and promoter haplotype diversity.

Saunders GC, Cawthraw S, Mountjoy SJ, Tout AC, Sayers AR, Hope J, Windl O.

J Gen Virol. 2009 May;90(Pt 5):1289-93. doi: 10.1099/vir.0.007997-0. Epub 2009 Mar 4.

PMID:
19264598
24.

Prion protein amino acid determinants of differential susceptibility and molecular feature of prion strains in mice and voles.

Agrimi U, Nonno R, Dell'Omo G, Di Bari MA, Conte M, Chiappini B, Esposito E, Di Guardo G, Windl O, Vaccari G, Lipp HP.

PLoS Pathog. 2008 Jul 25;4(7):e1000113. doi: 10.1371/journal.ppat.1000113.

25.

Evidence for a pathogenic role of different mutations at codon 188 of PRNP.

Roeber S, Grasbon-Frodl EM, Windl O, Krebs B, Xiang W, Vollmert C, Illig T, Schröter A, Arzberger T, Weber P, Zerr I, Kretzschmar HA.

PLoS One. 2008 May 14;3(5):e2147. doi: 10.1371/journal.pone.0002147. Erratum in: PLoS ONE. 2008;3(5). doi: 10.1371/annotation/3189bbdc-e736-4cb5-b1f2-9a5fb49536c6.

26.

Transcriptome analysis reveals altered cholesterol metabolism during the neurodegeneration in mouse scrapie model.

Xiang W, Hummel M, Mitteregger G, Pace C, Windl O, Mansmann U, Kretzschmar HA.

J Neurochem. 2007 Aug;102(3):834-47. Epub 2007 Apr 16.

27.

Polymorphisms of the prion protein gene coding region in born-after-the-reinforced-ban (BARB) bovine spongiform encephalopathy cattle in Great Britain.

Saunders GC, Griffiths PC, Cawthraw S, Tout AC, Wiener P, Woolliams JA, Williams JL, Windl O.

J Gen Virol. 2007 Apr;88(Pt 4):1374-8.

PMID:
17374784
28.

Identification of a proteinase K resistant protein for use as an internal positive control marker in PrP Western blotting.

Saunders GC, Horigan V, Tout AC, Windl O.

Res Vet Sci. 2007 Oct;83(2):157-64. Epub 2007 Mar 2.

PMID:
17336356
29.

Significant association of a M129V independent polymorphism in the 5' UTR of the PRNP gene with sporadic Creutzfeldt-Jakob disease in a large German case-control study.

Vollmert C, Windl O, Xiang W, Rosenberger A, Zerr I, Wichmann HE, Bickeböller H, Illig T; KORA group, Kretzschmar HA.

J Med Genet. 2006 Oct;43(10):e53.

30.

PrP genotypes of atypical scrapie cases in Great Britain.

Saunders GC, Cawthraw S, Mountjoy SJ, Hope J, Windl O.

J Gen Virol. 2006 Nov;87(Pt 11):3141-9.

PMID:
17030846
31.

Conversion efficiency of bank vole prion protein in vitro is determined by residues 155 and 170, but does not correlate with the high susceptibility of bank voles to sheep scrapie in vivo.

Piening N, Nonno R, Di Bari M, Walter S, Windl O, Agrimi U, Kretzschmar HA, Bertsch U.

J Biol Chem. 2006 Apr 7;281(14):9373-84. Epub 2006 Feb 2.

32.

Breaking an absolute species barrier: transgenic mice expressing the mink PrP gene are susceptible to transmissible mink encephalopathy.

Windl O, Buchholz M, Neubauer A, Schulz-Schaeffer W, Groschup M, Walter S, Arendt S, Neumann M, Voss AK, Kretzschmar HA.

J Virol. 2005 Dec;79(23):14971-5.

33.

Cerebral gene expression profiles in sporadic Creutzfeldt-Jakob disease.

Xiang W, Windl O, Westner IM, Neumann M, Zerr I, Lederer RM, Kretzschmar HA.

Ann Neurol. 2005 Aug;58(2):242-57.

PMID:
16049922
34.

Clinical course in young patients with sporadic Creutzfeldt-Jakob disease.

Boesenberg C, Schulz-Schaeffer WJ, Meissner B, Kallenberg K, Bartl M, Heinemann U, Krasnianski A, Stoeck K, Varges D, Windl O, Kretzschmar HA, Zerr I.

Ann Neurol. 2005 Oct;58(4):533-43.

PMID:
16037975
35.

Identification of the pathological prion protein allotypes in scrapie-infected heterozygous bank voles (Clethrionomys glareolus) by high-performance liquid chromatography-mass spectrometry.

Cartoni C, Schininà ME, Maras B, Nonno R, Vaccari G, Di Baria MA, Conte M, Liu QG, Lu M, Cardone F, Windl O, Pocchiari M, Agrimi U.

J Chromatogr A. 2005 Jul 15;1081(1):122-6.

PMID:
16013608
36.

Activation of phosphatidylinositol 3-kinase by cellular prion protein and its role in cell survival.

Vassallo N, Herms J, Behrens C, Krebs B, Saeki K, Onodera T, Windl O, Kretzschmar HA.

Biochem Biophys Res Commun. 2005 Jun 24;332(1):75-82.

PMID:
15896301
37.

Creutzfeldt-Jakob disease associated with an R148H mutation of the prion protein gene.

Krebs B, Lederer RM, Windl O, Grasbon-Frodl EM, Zerr I, Kretzschmar HA.

Neurogenetics. 2005 May;6(2):97-100. Epub 2005 Mar 18. No abstract available.

PMID:
15776279
38.

Creutzfeldt-Jakob disease in a patient with an R208H mutation of the prion protein gene (PRNP) and a 17-kDa prion protein fragment.

Roeber S, Krebs B, Neumann M, Windl O, Zerr I, Grasbon-Frodl EM, Kretzschmar HA.

Acta Neuropathol. 2005 Apr;109(4):443-8. Epub 2005 Mar 1.

PMID:
15739100
39.

Loss of glycosylation associated with the T183A mutation in human prion disease.

Grasbon-Frodl E, Lorenz H, Mann U, Nitsch RM, Windl O, Kretzschmar HA.

Acta Neuropathol. 2004 Dec;108(6):476-84. Epub 2004 Oct 26.

PMID:
15558291
40.

A novel three extra-repeat insertion in the prion protein gene (PRNP) in a patient with Creutzfeldt-Jakob disease.

Grasbon-Frodl E, Schmalzbauer R, Weber P, Krebs B, Windl O, Zerr I, Kretzschmar HA.

Neurogenetics. 2004 Dec;5(4):249-50. No abstract available.

PMID:
15480878
41.

Genetic mapping of activity determinants within cellular prion proteins: N-terminal modules in PrPC offset pro-apoptotic activity of the Doppel helix B/B' region.

Drisaldi B, Coomaraswamy J, Mastrangelo P, Strome B, Yang J, Watts JC, Chishti MA, Marvi M, Windl O, Ahrens R, Major F, Sy MS, Kretzschmar H, Fraser PE, Mount HT, Westaway D.

J Biol Chem. 2004 Dec 31;279(53):55443-54. Epub 2004 Sep 29.

42.

Identification of differentially expressed genes in scrapie-infected mouse brains by using global gene expression technology.

Xiang W, Windl O, Wünsch G, Dugas M, Kohlmann A, Dierkes N, Westner IM, Kretzschmar HA.

J Virol. 2004 Oct;78(20):11051-60.

43.

Sporadic Creutzfeldt-Jakob disease: magnetic resonance imaging and clinical findings.

Meissner B, Körtner K, Bartl M, Jastrow U, Mollenhauer B, Schröter A, Finkenstaedt M, Windl O, Poser S, Kretzschmar HA, Zerr I.

Neurology. 2004 Aug 10;63(3):450-6.

PMID:
15314808
44.

Early age of onset in fatal familial insomnia. Two novel cases and review of the literature.

Harder A, Gregor A, Wirth T, Kreuz F, Schulz-Schaeffer WJ, Windl O, Plotkin M, Amthauer H, Neukirch K, Kretzschmar HA, Kuhlmann T, Braas R, Hahne HH, Jendroska K.

J Neurol. 2004 Jun;251(6):715-24.

PMID:
15311348
45.

Efficacy of flupirtine on cognitive function in patients with CJD: A double-blind study.

Otto M, Cepek L, Ratzka P, Doehlinger S, Boekhoff I, Wiltfang J, Irle E, Pergande G, Ellers-Lenz B, Windl O, Kretzschmar HA, Poser S, Prange H.

Neurology. 2004 Mar 9;62(5):714-8.

PMID:
15007119
46.

Latrogenic Creutzfeldt-Jakob disease with florid plaques.

Kretzschmar HA, Sethi S, Földvári Z, Windl O, Querner V, Zerr I, Poser S.

Brain Pathol. 2003 Jul;13(3):245-9. Review.

PMID:
12946015
47.

Clinical findings in sporadic Creutzfeldt-Jakob disease correlate with thalamic pathology.

Tschampa HJ, Herms JW, Schulz-Schaeffer WJ, Maruschak B, Windl O, Jastrow U, Zerr I, Steinhoff BJ, Poser S, Kretzschmar HA.

Brain. 2002 Nov;125(Pt 11):2558-66.

PMID:
12390980
48.

Mutant prion protein acquires resistance to protease in mouse neuroblastoma cells.

Wegner C, Römer A, Schmalzbauer R, Lorenz H, Windl O, Kretzschmar HA.

J Gen Virol. 2002 May;83(Pt 5):1237-45.

PMID:
11961279
49.

[Clinically atypical CJD: diagnostic relevance of cerebrospinal fluid markers and molecular genetic analysis?].

Tumani H, Windl O, Kretzschmar HA, Ludolph AC.

Dtsch Med Wochenschr. 2002 Feb 15;127(7):318-20. German.

PMID:
11845387
50.

Cellular phenotyping of secretory and nuclear prion proteins associated with inherited prion diseases.

Lorenz H, Windl O, Kretzschmar HA.

J Biol Chem. 2002 Mar 8;277(10):8508-16. Epub 2001 Dec 27.

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