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Variant Creutzfeldt-Jakob disease strain is identical in individuals of two PRNP codon 129 genotypes.

Diack AB, Boyle A, Plinston C, Hunt E, Bishop MT, Will RG, Manson JC.

Brain. 2019 May 1;142(5):1416-1428. doi: 10.1093/brain/awz076. Erratum in: Brain. 2019 May 1;142(5):e22.


Unravelling the glial response in the pathogenesis of Alzheimer's disease.

Alibhai JD, Diack AB, Manson JC.

FASEB J. 2018 Nov;32(11):5766-5777. doi: 10.1096/fj.201801360R. Review.


Oral Prion Neuroinvasion Occurs Independently of PrPC Expression in the Gut Epithelium.

Marshall A, Bradford BM, Clarke AR, Manson JC, Mabbott NA.

J Virol. 2018 Sep 12;92(19). pii: e01010-18. doi: 10.1128/JVI.01010-18. Print 2018 Oct 1.


Spermine increases acetylation of tubulins and facilitates autophagic degradation of prion aggregates.

Phadwal K, Kurian D, Salamat MKF, MacRae VE, Diack AB, Manson JC.

Sci Rep. 2018 Jul 3;8(1):10004. doi: 10.1038/s41598-018-28296-y.


Public health risks from subclinical variant CJD.

Diack AB, Will RG, Manson JC.

PLoS Pathog. 2017 Nov 30;13(11):e1006642. doi: 10.1371/journal.ppat.1006642. eCollection 2017 Nov. Review. No abstract available.


Strain Typing of Prion Diseases Using In Vivo Mouse Models.

Boyle A, Hogan K, Manson JC, Diack AB.

Methods Mol Biol. 2017;1658:263-283. doi: 10.1007/978-1-4939-7244-9_18. Review.


Conditional Deletion of Prnp Rescues Behavioral and Synaptic Deficits after Disease Onset in Transgenic Alzheimer's Disease.

Salazar SV, Gallardo C, Kaufman AC, Herber CS, Haas LT, Robinson S, Manson JC, Lee MK, Strittmatter SM.

J Neurosci. 2017 Sep 20;37(38):9207-9221. doi: 10.1523/JNEUROSCI.0722-17.2017. Epub 2017 Aug 21.


Gene Targeted Transgenic Mouse Models in Prion Research.

Diack AB, Alibhai JD, Manson JC.

Prog Mol Biol Transl Sci. 2017;150:157-179. doi: 10.1016/bs.pmbts.2017.06.008. Epub 2017 Aug 2. Review.


Similarities of Variant Creutzfeldt-Jakob Disease Strain in Mother and Son in Spain to UK Reference Case.

Diack AB, Boyle A, Ritchie D, Plinston C, Kisielewski D, de Pedro-Cuesta J, Rábano A, Will RG, Manson JC.

Emerg Infect Dis. 2017 Sep;23(9):1593-1596. doi: 10.3201/eid2309.170159.


Pro-death NMDA receptor signaling is promoted by the GluN2B C-terminus independently of Dapk1.

McQueen J, Ryan TJ, McKay S, Marwick K, Baxter P, Carpanini SM, Wishart TM, Gillingwater TH, Manson JC, Wyllie DJA, Grant SGN, McColl BW, Komiyama NH, Hardingham GE.

Elife. 2017 Jul 21;6. pii: e17161. doi: 10.7554/eLife.17161.


Analysis of gene expression in the nervous system identifies key genes and novel candidates for health and disease.

Carpanini SM, Wishart TM, Gillingwater TH, Manson JC, Summers KM.

Neurogenetics. 2017 Apr;18(2):81-95. doi: 10.1007/s10048-017-0509-5. Epub 2017 Feb 11.


Sideroflexin 3 is an α-synuclein-dependent mitochondrial protein that regulates synaptic morphology.

Amorim IS, Graham LC, Carter RN, Morton NM, Hammachi F, Kunath T, Pennetta G, Carpanini SM, Manson JC, Lamont DJ, Wishart TM, Gillingwater TH.

J Cell Sci. 2017 Jan 15;130(2):325-331. doi: 10.1242/jcs.194241. Epub 2017 Jan 3.


Distribution of Misfolded Prion Protein Seeding Activity Alone Does Not Predict Regions of Neurodegeneration.

Alibhai J, Blanco RA, Barria MA, Piccardo P, Caughey B, Perry VH, Freeman TC, Manson JC.

PLoS Biol. 2016 Nov 23;14(11):e1002579. doi: 10.1371/journal.pbio.1002579. eCollection 2016 Nov.


Ablation of Prion Protein in Wild Type Human Amyloid Precursor Protein (APP) Transgenic Mice Does Not Alter The Proteolysis of APP, Levels of Amyloid-β or Pathologic Phenotype.

Whitehouse IJ, Brown D, Baybutt H, Diack AB, Kellett KA, Piccardo P, Manson JC, Hooper NM.

PLoS One. 2016 Jul 22;11(7):e0159119. doi: 10.1371/journal.pone.0159119. eCollection 2016.


Insights into Mechanisms of Chronic Neurodegeneration.

Diack AB, Alibhai JD, Barron R, Bradford B, Piccardo P, Manson JC.

Int J Mol Sci. 2016 Jan 12;17(1). pii: E82. doi: 10.3390/ijms17010082. Review.


Defining the Microglia Response during the Time Course of Chronic Neurodegeneration.

Vincenti JE, Murphy L, Grabert K, McColl BW, Cancellotti E, Freeman TC, Manson JC.

J Virol. 2015 Dec 30;90(6):3003-17. doi: 10.1128/JVI.02613-15.


A prion reduction filter does not completely remove endogenous prion infectivity from sheep blood.

McCutcheon S, Alejo Blanco AR, Tan BC, González L, Martin S, Mallinson G, Appleford NE, Turner ML, Manson JC, Houston EF.

Transfusion. 2015 Sep;55(9):2123-33. doi: 10.1111/trf.13145. Epub 2015 May 30.


The glycosylation status of PrPC is a key factor in determining transmissible spongiform encephalopathy transmission between species.

Wiseman FK, Cancellotti E, Piccardo P, Iremonger K, Boyle A, Brown D, Ironside JW, Manson JC, Diack AB.

J Virol. 2015 May;89(9):4738-47. doi: 10.1128/JVI.02296-14. Epub 2015 Feb 11.


Variant CJD. 18 years of research and surveillance.

Diack AB, Head MW, McCutcheon S, Boyle A, Knight R, Ironside JW, Manson JC, Will RG.

Prion. 2014;8(4):286-95. doi: 10.4161/pri.29237. Epub 2014 Nov 1. Review.


Variably protease-sensitive prionopathy, a unique prion variant with inefficient transmission properties.

Diack AB, Ritchie DL, Peden AH, Brown D, Boyle A, Morabito L, Maclennan D, Burgoyne P, Jansen C, Knight RS, Piccardo P, Ironside JW, Manson JC.

Emerg Infect Dis. 2014 Dec;20(12):1969-79. doi: 10.3201/eid2012.140214.


Increased susceptibility of transgenic mice expressing human PrP to experimental sheep bovine spongiform encephalopathy is not due to increased agent titre in sheep brain tissue.

Plinston C, Hart P, Hunter N, Manson JC, Barron RM.

J Gen Virol. 2014 Aug;95(Pt 8):1855-9. doi: 10.1099/vir.0.065730-0. Epub 2014 May 14.


Dissociation of prion protein amyloid seeding from transmission of a spongiform encephalopathy.

Piccardo P, King D, Telling G, Manson JC, Barron RM.

J Virol. 2013 Nov;87(22):12349-56. doi: 10.1128/JVI.00673-13. Epub 2013 Sep 11.


Prion infectivity in the spleen of a PRNP heterozygous individual with subclinical variant Creutzfeldt-Jakob disease.

Bishop MT, Diack AB, Ritchie DL, Ironside JW, Will RG, Manson JC.

Brain. 2013 Apr;136(Pt 4):1139-45. doi: 10.1093/brain/awt032. Epub 2013 Feb 28.


Post-translational changes to PrP alter transmissible spongiform encephalopathy strain properties.

Cancellotti E, Mahal SP, Somerville R, Diack A, Brown D, Piccardo P, Weissmann C, Manson JC.

EMBO J. 2013 Mar 6;32(5):756-69. doi: 10.1038/emboj.2013.6. Epub 2013 Feb 8.


Constant transmission properties of variant Creutzfeldt-Jakob disease in 5 countries.

Diack AB, Ritchie D, Bishop M, Pinion V, Brandel JP, Haik S, Tagliavini F, Van Duijn C, Belay ED, Gambetti P, Schonberger LB, Piccardo P, Will RG, Manson JC.

Emerg Infect Dis. 2012 Oct;18(10):1574-9. doi: 10.3201/eid1810.120792.


Significant differences in incubation times in sheep infected with bovine spongiform encephalopathy result from variation at codon 141 in the PRNP gene.

Tan BC, Blanco AR, Houston EF, Stewart P, Goldmann W, Gill AC, de Wolf C, Manson JC, McCutcheon S.

J Gen Virol. 2012 Dec;93(Pt 12):2749-56. doi: 10.1099/vir.0.039008-0. Epub 2012 Sep 12.


Chronic wasting disease and atypical forms of bovine spongiform encephalopathy and scrapie are not transmissible to mice expressing wild-type levels of human prion protein.

Wilson R, Plinston C, Hunter N, Casalone C, Corona C, Tagliavini F, Suardi S, Ruggerone M, Moda F, Graziano S, Sbriccoli M, Cardone F, Pocchiari M, Ingrosso L, Baron T, Richt J, Andreoletti O, Simmons M, Lockey R, Manson JC, Barron RM.

J Gen Virol. 2012 Jul;93(Pt 7):1624-9. doi: 10.1099/vir.0.042507-0. Epub 2012 Apr 11.


Cellular prion protein expression is not regulated by the Alzheimer's amyloid precursor protein intracellular domain.

Lewis V, Whitehouse IJ, Baybutt H, Manson JC, Collins SJ, Hooper NM.

PLoS One. 2012;7(2):e31754. doi: 10.1371/journal.pone.0031754. Epub 2012 Feb 21.


MM2-thalamic Creutzfeldt-Jakob disease: neuropathological, biochemical and transmission studies identify a distinctive prion strain.

Moda F, Suardi S, Di Fede G, Indaco A, Limido L, Vimercati C, Ruggerone M, Campagnani I, Langeveld J, Terruzzi A, Brambilla A, Zerbi P, Fociani P, Bishop MT, Will RG, Manson JC, Giaccone G, Tagliavini F.

Brain Pathol. 2012 Sep;22(5):662-9. doi: 10.1111/j.1750-3639.2012.00572.x. Epub 2012 Feb 21.


Follicular dendritic cell-specific prion protein (PrP) expression alone is sufficient to sustain prion infection in the spleen.

McCulloch L, Brown KL, Bradford BM, Hopkins J, Bailey M, Rajewsky K, Manson JC, Mabbott NA.

PLoS Pathog. 2011 Dec;7(12):e1002402. doi: 10.1371/journal.ppat.1002402. Epub 2011 Dec 1.


All clinically-relevant blood components transmit prion disease following a single blood transfusion: a sheep model of vCJD.

McCutcheon S, Alejo Blanco AR, Houston EF, de Wolf C, Tan BC, Smith A, Groschup MH, Hunter N, Hornsey VS, MacGregor IR, Prowse CV, Turner M, Manson JC.

PLoS One. 2011;6(8):e23169. doi: 10.1371/journal.pone.0023169. Epub 2011 Aug 17.


Prion protein interacts with BACE1 protein and differentially regulates its activity toward wild type and Swedish mutant amyloid precursor protein.

Griffiths HH, Whitehouse IJ, Baybutt H, Brown D, Kellett KA, Jackson CD, Turner AJ, Piccardo P, Manson JC, Hooper NM.

J Biol Chem. 2011 Sep 23;286(38):33489-500. doi: 10.1074/jbc.M111.278556. Epub 2011 Jul 27.


Mechanism of PrP-amyloid formation in mice without transmissible spongiform encephalopathy.

Jeffrey M, McGovern G, Chambers EV, King D, González L, Manson JC, Ghetti B, Piccardo P, Barron RM.

Brain Pathol. 2012 Jan;22(1):58-66. doi: 10.1111/j.1750-3639.2011.00508.x. Epub 2011 Jul 25.


A polymorphism in the regulatory region of PRNP is associated with increased risk of sporadic Creutzfeldt-Jakob disease.

Sanchez-Juan P, Bishop MT, Croes EA, Knight RS, Will RG, van Duijn CM, Manson JC.

BMC Med Genet. 2011 May 22;12:73. doi: 10.1186/1471-2350-12-73.


Human embryonic stem cells rapidly take up and then clear exogenous human and animal prions in vitro.

Krejciova Z, Pells S, Cancellotti E, Freile P, Bishop M, Samuel K, Barclay GR, Ironside JW, Manson JC, Turner ML, De Sousa P, Head MW.

J Pathol. 2011 Apr;223(5):635-45. doi: 10.1002/path.2832. Epub 2011 Feb 21.


Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy infection following passage in sheep.

Plinston C, Hart P, Chong A, Hunter N, Foster J, Piccardo P, Manson JC, Barron RM.

J Virol. 2011 Feb;85(3):1174-81. doi: 10.1128/JVI.01578-10. Epub 2010 Nov 17.


Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties.

Bishop MT, Will RG, Manson JC.

Proc Natl Acad Sci U S A. 2010 Jun 29;107(26):12005-10. doi: 10.1073/pnas.1004688107. Epub 2010 Jun 14.


Glycosylation of PrPC determines timing of neuroinvasion and targeting in the brain following transmissible spongiform encephalopathy infection by a peripheral route.

Cancellotti E, Bradford BM, Tuzi NL, Hickey RD, Brown D, Brown KL, Barron RM, Kisielewski D, Piccardo P, Manson JC.

J Virol. 2010 Apr;84(7):3464-75. doi: 10.1128/JVI.02374-09. Epub 2010 Jan 27.


Dramatic reduction of PrP C level and glycosylation in peripheral nerves following PrP knock-out from Schwann cells does not prevent transmissible spongiform encephalopathy neuroinvasion.

Bradford BM, Tuzi NL, Feltri ML, McCorquodale C, Cancellotti E, Manson JC.

J Neurosci. 2009 Dec 9;29(49):15445-54. doi: 10.1523/JNEUROSCI.4195-09.2009.


No major change in vCJD agent strain after secondary transmission via blood transfusion.

Bishop MT, Ritchie DL, Will RG, Ironside JW, Head MW, Thomson V, Bruce M, Manson JC.

PLoS One. 2008 Aug 6;3(8):e2878. doi: 10.1371/journal.pone.0002878.


Host PrP glycosylation: a major factor determining the outcome of prion infection.

Tuzi NL, Cancellotti E, Baybutt H, Blackford L, Bradford B, Plinston C, Coghill A, Hart P, Piccardo P, Barron RM, Manson JC.

PLoS Biol. 2008 Apr 15;6(4):e100. doi: 10.1371/journal.pbio.0060100.


High titers of transmissible spongiform encephalopathy infectivity associated with extremely low levels of PrPSc in vivo.

Barron RM, Campbell SL, King D, Bellon A, Chapman KE, Williamson RA, Manson JC.

J Biol Chem. 2007 Dec 7;282(49):35878-86. Epub 2007 Oct 8.


In vitro amplification and detection of variant Creutzfeldt-Jakob disease PrPSc.

Jones M, Peden AH, Prowse CV, Gröner A, Manson JC, Turner ML, Ironside JW, MacGregor IR, Head MW.

J Pathol. 2007 Sep;213(1):21-6.


Cellular prion protein regulates beta-secretase cleavage of the Alzheimer's amyloid precursor protein.

Parkin ET, Watt NT, Hussain I, Eckman EA, Eckman CB, Manson JC, Baybutt HN, Turner AJ, Hooper NM.

Proc Natl Acad Sci U S A. 2007 Jun 26;104(26):11062-7. Epub 2007 Jun 15.


Accumulation of prion protein in the brain that is not associated with transmissible disease.

Piccardo P, Manson JC, King D, Ghetti B, Barron RM.

Proc Natl Acad Sci U S A. 2007 Mar 13;104(11):4712-7. Epub 2007 Mar 6.


The role of host PrP in Transmissible Spongiform Encephalopathies.

Cancellotti E, Barron RM, Bishop MT, Hart P, Wiseman F, Manson JC.

Biochim Biophys Acta. 2007 Jun;1772(6):673-80. Epub 2006 Oct 26. Review.


A novel, resistance-linked ovine PrP variant and its equivalent mouse variant modulate the in vitro cell-free conversion of rPrP to PrP(res).

Kirby L, Goldmann W, Houston F, Gill AC, Manson JC.

J Gen Virol. 2006 Dec;87(Pt 12):3747-51.


The transmissible spongiform encephalopathies: emerging and declining epidemics.

Manson JC, Cancellotti E, Hart P, Bishop MT, Barron RM.

Biochem Soc Trans. 2006 Dec;34(Pt 6):1155-8. Review.


Predicting susceptibility and incubation time of human-to-human transmission of vCJD.

Bishop MT, Hart P, Aitchison L, Baybutt HN, Plinston C, Thomson V, Tuzi NL, Head MW, Ironside JW, Will RG, Manson JC.

Lancet Neurol. 2006 May;5(5):393-8.


Altered glycosylated PrP proteins can have different neuronal trafficking in brain but do not acquire scrapie-like properties.

Cancellotti E, Wiseman F, Tuzi NL, Baybutt H, Monaghan P, Aitchison L, Simpson J, Manson JC.

J Biol Chem. 2005 Dec 30;280(52):42909-18. Epub 2005 Oct 11.

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