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Renewed assessment of the risk of emergent advanced cell therapies to transmit neuroproteinopathies.

De Sousa PA, Ritchie D, Green A, Chandran S, Knight R, Head MW.

Acta Neuropathol. 2019 Mar;137(3):363-377. doi: 10.1007/s00401-018-1941-9. Epub 2018 Nov 27. Review.


Susceptibility of Human Prion Protein to Conversion by Chronic Wasting Disease Prions.

Barria MA, Libori A, Mitchell G, Head MW.

Emerg Infect Dis. 2018 Aug;24(8):1482-1489. doi: 10.3201/eid2408.161888.


Rapid amplification of prions from variant Creutzfeldt-Jakob disease cerebrospinal fluid.

Barria MA, Lee A, Green AJ, Knight R, Head MW.

J Pathol Clin Res. 2018 Apr;4(2):86-92. doi: 10.1002/cjp2.90. Epub 2018 Feb 5.


Human stem cell-derived astrocytes replicate human prions in a PRNP genotype-dependent manner.

Krejciova Z, Alibhai J, Zhao C, Krencik R, Rzechorzek NM, Ullian EM, Manson J, Ironside JW, Head MW, Chandran S.

J Exp Med. 2017 Dec 4;214(12):3481-3495. doi: 10.1084/jem.20161547. Epub 2017 Nov 15.


Prion diseases.

Ironside JW, Ritchie DL, Head MW.

Handb Clin Neurol. 2017;145:393-403. doi: 10.1016/B978-0-12-802395-2.00028-6. Review.


Distribution and Quantitative Estimates of Variant Creutzfeldt-Jakob Disease Prions in Tissues of Clinical and Asymptomatic Patients.

Douet JY, Lacroux C, Aron N, Head MW, Lugan S, Tillier C, Huor A, Cassard H, Arnold M, Beringue V, Ironside JW, Andréoletti O.

Emerg Infect Dis. 2017 Jun;23(6):946-956. doi: 10.3201/eid2306.161734.


Amyloid-β accumulation in the CNS in human growth hormone recipients in the UK.

Ritchie DL, Adlard P, Peden AH, Lowrie S, Le Grice M, Burns K, Jackson RJ, Yull H, Keogh MJ, Wei W, Chinnery PF, Head MW, Ironside JW.

Acta Neuropathol. 2017 Aug;134(2):221-240. doi: 10.1007/s00401-017-1703-0. Epub 2017 Mar 27.


UK Iatrogenic Creutzfeldt-Jakob disease: investigating human prion transmission across genotypic barriers using human tissue-based and molecular approaches.

Ritchie DL, Barria MA, Peden AH, Yull HM, Kirkpatrick J, Adlard P, Ironside JW, Head MW.

Acta Neuropathol. 2017 Apr;133(4):579-595. doi: 10.1007/s00401-016-1638-x. Epub 2016 Nov 3.


Relative Abundance of apoE and Aβ1-42 Associated with Abnormal Prion Protein Differs between Creutzfeldt-Jakob Disease Subtypes.

Moore RA, Choi YP, Head MW, Ironside JW, Faris R, Ritchie DL, Zanusso G, Priola SA.

J Proteome Res. 2016 Dec 2;15(12):4518-4531. Epub 2016 Sep 30.


Correction: The Distribution of Prion Protein Allotypes Differs Between Sporadic and Iatrogenic Creutzfeldt-Jakob Disease Patients.

Moore RA, Head MW, Ironside JW, Ritchie DL, Zanusso G, Choi YP, Priola SA.

PLoS Pathog. 2016 Mar 8;12(3):e1005496. doi: 10.1371/journal.ppat.1005496. eCollection 2016 Mar. No abstract available.


The Distribution of Prion Protein Allotypes Differs Between Sporadic and Iatrogenic Creutzfeldt-Jakob Disease Patients.

Moore RA, Head MW, Ironside JW, Ritchie DL, Zanusso G, Choi YP, Priola SA.

PLoS Pathog. 2016 Feb 3;12(2):e1005416. doi: 10.1371/journal.ppat.1005416. eCollection 2016 Feb. Erratum in: PLoS Pathog. 2016 Mar;12(3):e1005496. Pyo Choi, Young [corrected to Choi, Young Pyo].


A case of variably protease-sensitive prionopathy treated with doxycyclin.

Assar H, Topakian R, Weis S, Rahimi J, Trenkler J, Höftberger R, Aboulenein-Djamshidian F, Ströbel T, Budka H, Yull H, Head MW, Ironside JW, Kovacs GG.

J Neurol Neurosurg Psychiatry. 2015 Jul;86(7):816-8. doi: 10.1136/jnnp-2014-309871. Epub 2015 Jan 9. No abstract available.


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.


Pathological and biochemical investigation of a woman diagnosed with genetic Creutzfeldt-Jakob disease shortly after parturition.

Head MW, Yull HM, Törö K, Keller E, Rozsa C, Ironside JW, Kovacs GG.

Neuropathol Appl Neurobiol. 2015 Aug;41(5):676-80. doi: 10.1111/nan.12204. Epub 2015 May 2. No abstract available.


The prion protein protease sensitivity, stability and seeding activity in variably protease sensitive prionopathy brain tissue suggests molecular overlaps with sporadic Creutzfeldt-Jakob disease.

Peden AH, Sarode DP, Mulholland CR, Barria MA, Ritchie DL, Ironside JW, Head MW.

Acta Neuropathol Commun. 2014 Oct 21;2:152. doi: 10.1186/s40478-014-0152-4.


Uptake and degradation of protease-sensitive and -resistant forms of abnormal human prion protein aggregates by human astrocytes.

Choi YP, Head MW, Ironside JW, Priola SA.

Am J Pathol. 2014 Dec;184(12):3299-307. doi: 10.1016/j.ajpath.2014.08.005. Epub 2014 Sep 30.


Genotype-dependent molecular evolution of sheep bovine spongiform encephalopathy (BSE) prions in vitro affects their zoonotic potential.

Krejciova Z, Barria MA, Jones M, Ironside JW, Jeffrey M, González L, Head MW.

J Biol Chem. 2014 Sep 19;289(38):26075-88. doi: 10.1074/jbc.M114.582965. Epub 2014 Aug 6.


Gerstmann-Straüssler-Scheinker disease: novel PRNP mutation and VGKC-complex antibodies.

Jones M, Odunsi S, du Plessis D, Vincent A, Bishop M, Head MW, Ironside JW, Gow D.

Neurology. 2014 Jun 10;82(23):2107-11. doi: 10.1212/WNL.0000000000000500. Epub 2014 May 9.


Exploring the zoonotic potential of animal prion diseases: in vivo and in vitro approaches.

Barria MA, Ironside JW, Head MW.

Prion. 2014 Jan-Feb;8(1):85-91.


Molecular barriers to zoonotic transmission of prions.

Barria MA, Balachandran A, Morita M, Kitamoto T, Barron R, Manson J, Knight R, Ironside JW, Head MW.

Emerg Infect Dis. 2014 Jan;20(1):88-97. doi: 10.3201/eid2001.130858.


Rapidly progressive dementia with thalamic degeneration and peculiar cortical prion protein immunoreactivity, but absence of proteinase K resistant PrP: a new disease entity?

Kovacs GG, Peden A, Weis S, Höftberger R, Berghoff AS, Yull H, Ströbel T, Koppi S, Katzenschlager R, Langenscheidt D, Assar H, Zaruba E, Gröner A, Voigtländer T, Puska G, Hametner E, Grams A, Muigg A, Knoflach M, László L, Ironside JW, Head MW, Budka H.

Acta Neuropathol Commun. 2013 Nov 11;1:72. doi: 10.1186/2051-5960-1-72.


Human tonsil-derived follicular dendritic-like cells are refractory to human prion infection in vitro and traffic disease-associated prion protein to lysosomes.

Krejciova Z, De Sousa P, Manson J, Ironside JW, Head MW.

Am J Pathol. 2014 Jan;184(1):64-70. doi: 10.1016/j.ajpath.2013.09.013. Epub 2013 Nov 1.


Variably protease-sensitive prionopathy in the UK: a retrospective review 1991-2008.

Head MW, Yull HM, Ritchie DL, Langeveld JP, Fletcher NA, Knight RS, Ironside JW.

Brain. 2013 Apr;136(Pt 4):1102-15. doi: 10.1093/brain/aws366. Review. Erratum in: Brain. 2013 Nov;136(Pt 11):i1.


Human prion diseases: molecular, cellular and population biology.

Head MW.

Neuropathology. 2013 Jun;33(3):221-36. doi: 10.1111/neup.12016. Epub 2013 Jan 20. Review.


Real time quaking-induced conversion analysis of cerebrospinal fluid in sporadic Creutzfeldt-Jakob disease.

McGuire LI, Peden AH, Orrú CD, Wilham JM, Appleford NE, Mallinson G, Andrews M, Head MW, Caughey B, Will RG, Knight RS, Green AJ.

Ann Neurol. 2012 Aug;72(2):278-85. doi: 10.1002/ana.23589.


Review: Creutzfeldt-Jakob disease: prion protein type, disease phenotype and agent strain.

Head MW, Ironside JW.

Neuropathol Appl Neurobiol. 2012 Jun;38(4):296-310. doi: 10.1111/j.1365-2990.2012.01265.x. Review.


The contribution of different prion protein types and host polymorphisms to clinicopathological variations in Creutzfeldt-Jakob disease.

Head MW, Ironside JW.

Rev Med Virol. 2012 Jul;22(4):214-29. doi: 10.1002/rmv.725. Epub 2012 Feb 28. Review.


Sensitive and specific detection of sporadic Creutzfeldt-Jakob disease brain prion protein using real-time quaking-induced conversion.

Peden AH, McGuire LI, Appleford NE, Mallinson G, Wilham JM, Orrú CD, Caughey B, Ironside JW, Knight RS, Will RG, Green AJ, Head MW.

J Gen Virol. 2012 Feb;93(Pt 2):438-49. doi: 10.1099/vir.0.033365-0. Epub 2011 Oct 26.


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.


RNA integrity in post mortem human variant Creutzfeldt-Jakob disease (vCJD) and control brain tissue.

Sherwood KR, Head MW, Walker R, Smith C, Ironside JW, Fazakerley JK.

Neuropathol Appl Neurobiol. 2011 Oct;37(6):633-42. doi: 10.1111/j.1365-2990.2011.01162.x.


Variably protease-sensitive prionopathy in a PRNP codon 129 heterozygous UK patient with co-existing tau, α synuclein and Aβ pathology.

Head MW, Lowrie S, Chohan G, Knight R, Scoones DJ, Ironside JW.

Acta Neuropathol. 2010 Dec;120(6):821-3. doi: 10.1007/s00401-010-0766-y. Epub 2010 Nov 3. No abstract available.


Correlation of polydispersed prion protein and characteristic pathology in the thalamus in variant Creutzfeldt-Jakob disease: implication of small oligomeric species.

Choi YP, Gröner A, Ironside JW, Head MW.

Brain Pathol. 2011 May;21(3):298-307. doi: 10.1111/j.1750-3639.2010.00446.x. Epub 2010 Nov 3.


Distinct stability states of disease-associated human prion protein identified by conformation-dependent immunoassay.

Choi YP, Peden AH, Gröner A, Ironside JW, Head MW.

J Virol. 2010 Nov;84(22):12030-8. doi: 10.1128/JVI.01057-10. Epub 2010 Sep 15.


The first case of protease-sensitive prionopathy (PSPr) in The Netherlands: a patient with an unusual GSS-like clinical phenotype.

Jansen C, Head MW, van Gool WA, Baas F, Yull H, Ironside JW, Rozemuller AJ.

J Neurol Neurosurg Psychiatry. 2010 Sep;81(9):1052-5. doi: 10.1136/jnnp.2009.175646. Epub 2010 Jun 14.


The application of in vitro cell-free conversion systems to human prion diseases.

Jones M, Peden AH, Head MW, Ironside JW.

Acta Neuropathol. 2011 Jan;121(1):135-43. doi: 10.1007/s00401-010-0708-8. Epub 2010 Jun 10. Review.


A novel seven-octapeptide repeat insertion in the prion protein gene (PRNP) in a Dutch pedigree with Gerstmann-Sträussler-Scheinker disease phenotype: comparison with similar cases from the literature.

Jansen C, Voet W, Head MW, Parchi P, Yull H, Verrips A, Wesseling P, Meulstee J, Baas F, van Gool WA, Ironside JW, Rozemuller AJ.

Acta Neuropathol. 2011 Jan;121(1):59-68. doi: 10.1007/s00401-010-0656-3. Epub 2010 Mar 3. Review.


Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia.

Peden A, McCardle L, Head MW, Love S, Ward HJ, Cousens SN, Keeling DM, Millar CM, Hill FG, Ironside JW.

Haemophilia. 2010 Mar;16(2):296-304. doi: 10.1111/j.1365-2516.2009.02181.x. Epub 2010 Jan 12.


Molecular model of prion transmission to humans.

Jones M, Wight D, Barron R, Jeffrey M, Manson J, Prowse C, Ironside JW, Head MW.

Emerg Infect Dis. 2009 Dec;15(12):2013-6. doi: 10.3201/eid1512.090194.


Differential protein profiling as a potential multi-marker approach for TSE diagnosis.

Barr JB, Watson M, Head MW, Ironside JW, Harris N, Hogarth C, Fraser JR, Barron R.

BMC Infect Dis. 2009 Nov 27;9:188. doi: 10.1186/1471-2334-9-188.


Sporadic Creutzfeldt-Jakob disease: discrete subtypes or a spectrum of disease?

Head MW, Ironside JW.

Brain. 2009 Oct;132(Pt 10):2627-9. doi: 10.1093/brain/awp225. Epub 2009 Sep 16. No abstract available.


A case of protease sensitive prionopathy in a patient in the UK.

Head MW, Knight R, Zeidler M, Yull H, Barlow A, Ironside JW.

Neuropathol Appl Neurobiol. 2009 Dec;35(6):628-32. doi: 10.1111/j.1365-2990.2009.01040.x. Epub 2009 Aug 7. No abstract available.


Transmissions of variant Creutzfeldt-Jakob disease from brain and lymphoreticular tissue show uniform and conserved bovine spongiform encephalopathy-related phenotypic properties on primary and secondary passage in wild-type mice.

Ritchie DL, Boyle A, McConnell I, Head MW, Ironside JW, Bruce ME.

J Gen Virol. 2009 Dec;90(Pt 12):3075-82. doi: 10.1099/vir.0.013227-0. Epub 2009 Aug 5.


Pathological investigation of the first blood donor and recipient pair linked by transfusion-associated variant Creutzfeldt-Jakob disease transmission.

Head MW, Yull HM, Ritchie DL, Bishop MT, Ironside JW.

Neuropathol Appl Neurobiol. 2009 Aug;35(4):433-6. doi: 10.1111/j.1365-2990.2009.01025.x. Epub 2009 Apr 7. No abstract available.


Panencephalopathic Creutzfeldt-Jakob disease in the Netherlands and the UK: clinical and pathological characteristics of nine patients.

Jansen C, Head MW, Rozemuller AJ, Ironside JW.

Neuropathol Appl Neurobiol. 2009 Jun;35(3):272-82. doi: 10.1111/j.1365-2990.2008.01004a.x.


Variant Creutzfeldt-Jakob disease in France and the United Kingdom: Evidence for the same agent strain.

Brandel JP, Heath CA, Head MW, Levavasseur E, Knight R, Laplanche JL, Langeveld JP, Ironside JW, Hauw JJ, Mackenzie J, Alpérovitch A, Will RG, Haïk S.

Ann Neurol. 2009 Mar;65(3):249-56. doi: 10.1002/ana.21583.


Further characterisation of the prion protein molecular types detectable in the NIBSC Creutzfeldt-Jakob disease brain reference materials.

Yull HM, Ironside JW, Head MW.

Biologicals. 2009 Aug;37(4):210-5. doi: 10.1016/j.biologicals.2009.01.009. Epub 2009 Mar 4.


Familial prion disease with a novel serine to isoleucine mutation at codon 132 of prion protein gene (PRNP).

Hilton DA, Head MW, Singh VK, Bishop M, Ironside JW.

Neuropathol Appl Neurobiol. 2009 Feb;35(1):111-5. doi: 10.1111/j.1365-2990.2008.00964.x. No abstract available.


Production and characterization of a panel of monoclonal antibodies against native human cellular prion protein.

Jones M, McLoughlin V, Connolly JG, Farquhar CF, MacGregor IR, Head MW.

Hybridoma (Larchmt). 2009 Feb;28(1):13-20. doi: 10.1089/hyb.2008.0067.


Human platelets as a substrate source for the in vitro amplification of the abnormal prion protein (PrP) associated with variant Creutzfeldt-Jakob disease.

Jones M, Peden AH, Yull H, Wight D, Bishop MT, Prowse CV, Turner ML, Ironside JW, MacGregor IR, Head MW.

Transfusion. 2009 Feb;49(2):376-84. doi: 10.1111/j.1537-2995.2008.01954.x. Epub 2008 Oct 29.


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