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MRC Prion Unit
From fundamental research to prevention and cure

2010 Publications

Prion strain mutation and selection
Collinge J. Science 2010; 328: 1111-2.

Contribution of transgenic models to understanding human prion disease
Wadsworth JDF, Asante EA, Collinge J.  Neuropathol App Nerobiol 2010; 36: 576-97.

A standardised comparison of commercially available prion decontamination reagents using the standard steel binding assay
Edgeworth JA, Sicilia A, Linehan J, Brandner S, Jackson GS, Collinge J.
J Gen Virol. 2010 Nov 17.

The retinoic acid receptor beta (Rarb) region of Mmu14 is associated with prion disease incubation time in mouse.
Grizenkova J, Akhtar S, Collinge J, Lloyd SE. PLoS One 2010; 5: e15019. 

The legs at odd angles (Loa) mutation incytoplasmic dynein ameliorates mitochondrial function in SOD1G93A mouse modelfor motor neuron disease
El Kadi AM, Bros-Facer V, Deng W, Philpott A, Stoddart E, Banks G, Jackson GS, Fisher EM, Duchen MR, Greensmith L, Moore AL, Hafezparast M.  J Biol Chem 2010; 285: 18627-39.

Genetic evidence implicates the immune system and cholesterol metabolism in the aetiology of
Alzheimer's disease

Jones L, Holmans PA, Hamshere ML, Harold D, Moskvina V, Ivanov D, Pocklington A, Abraham R, Hollingworth P, Sims R, Gerrish A, Pahwa JS, Jones N, Stretton A, Morgan AR, Lovestone S, Powell J, Proitsi P, Lupton MK, Brayne C, Rubinsztein DC, Gill M, Lawlor B, Lynch A, Morgan K, Brown KS, Passmore PA, Craig D, McGuinness B, Todd S, Holmes C, Mann D, Smith AD, Love S, Kehoe PG, Mead S, Fox N, Rossor M, Collinge J, Maier W, Jessen F, Schürmann B, van den Bussche H, Heuser I, Peters O, Kornhuber J, Wiltfang J, Dichgans M, Frölich L, Hampel H, Hüll M, Rujescu D, Goate AM, Kauwe JS, Cruchaga C, Nowotny P, Morris JC, Mayo K, Livingston G, Bass NJ, Gurling H, McQuillin A, Gwilliam R, Deloukas P, Al-Chalabi A, Shaw CE, Singleton AB, Guerreiro R, Mühleisen TW, Nöthen MM, Moebus S, Jöckel KH, Klopp N, Wichmann HE, Rüther E, Carrasquillo MM, Pankratz VS, Younkin SG, Hardy J, O'Donovan MC, Owen MJ, Williams J.  PLoS One 2010; 5: e13950.

Isolation of proteinase K-sensitive prions using pronase E and phosphotungstic acid
D’Castro L, Wenborn A, Gros N, Joiner S, Cronier S, Collinge J, Wadsworth JDF. PLoS One 2010; 5: e15679. 

Modification of superoxide dismutase 1 (SOD1) properties by a GFPtag--implications for research into amyotrophic lateral sclerosis (ALS)
Stevens JC, Chia R, Hendriks WT, Bros-Facer V, van Minnen J, Martin JE, Jackson GS, Greensmith L, Schiavo G, Fisher EM.  PLoS ONE 2010; 5: e9541.

Magnetization transfer ratio may be a surrogate of spongiform change in human prion diseases
Siddique D, Hyare H, Wroe S, Webb T, Macfarlane R, Rudge P, Collinge J, Powell C, Brandner S, So PW, Walker S, Mead S, Yousry T, Thornton JS.  Brain 2010; 133: 3058-68.

The H187R mutation of the human prion protein induces conversion of recombinant prion protein to PrPSc-like form
Hosszu L, Tattum H, Jones S, Trevitt CR, Wells MA, Waltho JP, Collinge J, Jackson GS, Clarke AR.
Biochemistry 2010; 49: 8729-38.

Amyloid beta-protein dimers rapidly form stable synaptotoxic protofibrils
O'Nuallain B, Freir DB, Nicoll AJ, Risse E, Ferguson N, Herron CE, Collinge J, Walsh DM.
J Neurosci 2010; 30: 14411-19.

Pharmacological chaperone for the structured domain of human prion protein
Nicoll A, Trevitt C, Risse E, Quaterman E, Ibarra AA, Wright C, Jackson GS, Sessions R, Farrow M, Waltho J, Clarke A, Collinge J.  Proc Natl Acad Sci USA 2010; 107; 17610-5.

Chronic wasting disease prions are not transmissible to transgenic mice over-expressing human prion protein
Sandberg MK, Al-Doujaily H, Sigurdson CJ, Glatzel M, O’Malley C, Powell C, Asante EA, Linehan JM, Brandner S, Wadsworth JDF, Collinge J.  J Gen Virol 2010; 91: 2651-7.

Spontaneous generation of mammalian prions
Edgeworth J, Gros N, Alden J, Joiner S, Wadsworth JDF, Linehan J, Brandner S, Jackson GS, Weissmann C, Collinge J.  Proc Natl Acad Sci USA 2010; 107: 14402-6.

FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration
Urwin H, Josephs KA, Rohrer JD, Authier A, Neumann M, Mackenzie IR, Seelaar H, Van Swieten JC, Brown JM, Johannsen P, Nielsen JE, Holm IE, The FReJA Consortium, Dickson DW, Rademakers R, Graff-Radford NR, Parisi JE, Petersen RC, Hatanpaa KJ, White III CL, Weiner MF, Van Deerlin VM, Trojanowski JQ, Miller BL, van der Zee J, Kumar-Singh S, Van Broeckhoven C, Bigio EH, Halliday GM, Kril JJ, Munoz DG, Mann DM, Pickering-Brown SM, Uphill J, Ghazi-Noori S, Fisher EMC, Holton JL, Revesz T, Rossor MN, Collinge J, Mead S. Isaacs AM.  Acta Neuropathol 2010; 120: 33-41.

Superoxide dismutase 1 and tgSOD1 mouse spinal cord seed fibrils, suggesting a propagative cell death mechanism in amyotrophic lateral sclerosis
Chia R, Tattum HM, Jones S, Collinge J, Fisher EMC, Jackson GS.  PlosOne 2010; 5: e10627.

A highly sensitive immunoassay for the detection of prion-infected material in whole human blood without the use of proteinase K.
Tattum MH, Jones S, Pal S, Khalili-Shirazi A, Collinge J, Jackson GS. Transfusion 2010; 50: 2619-27.

PRNP allelic series from 19 years of prion protein gene sequencing at the MRC Prion Unit
Beck JA, Poulter M, Campbell TA, Adamson G, Uphill JB, Guerreiro R, Stephens JC, Jackson GS, Manji H, Collinge J, Mead S.  Hum Mut 2010; 31: E1551- 63.

Discrimination between prioninfected and normal blood samples by protein misfolding cyclic amplification
Tattum MH, Jones S, Pal S, Collinge J, Jackson GS. Transfusion 2010; 50: 996-1002.

Genetic variability in CLU and its association with Alzheimer's disease
Guerreiro RJ, Beck J, Gibbs JR, Santana I, Rossor MN, Schott JM, Nalls MA, Ribeiro H, Santiago B, Fox NC, Oliveira C, Collinge J, Mead S, Singleton A, Hardy J.  PLoS One 2010; 5: e9510.

Disruption of endocytic trafficking in frontotemporal dementia with CHMP2B mutations
Urwin H, Authier A, Neilsen J, Powell C, Metcalf D, Froud K, Holm I, Johannsen P, Brown J, Fisher E, van der Zee J, Bruyland M, Van Broeckhoven C, Collinge J, Brander S, Futter C, Isaacs A.
Hum Mol Genet 2010; 19: 2228-38.

Heterozygosity at polymorphic codon 219 in variant Creutzfeldt-Jakob disease.
Lukic A, Beck JA, Joiner S, Fearnley J, Sturman S, Brandner S, Wadsworth JDF, Collinge J, Mead S.  Arch Neurol 2010; 67: 1021-3.

High-b-value diffusion mr imaging and basal nuclei apparent diffusion coefficient measurements in variant and sporadic Creutzfeldt-Jakob disease
Hyare H, Thornton J, Stevens J, Mead S, Rudge P, Collinge J, Yousry TA, Jäger HR.  Amer J Neuroradiol 2010; 31: 521-6.

Brain-water diffusion coefficients reflect the severity of inherited prion disease
Hyare H, Wroe SJ, Siddique D, Stevens J, Webb T, Fox N, Collinge J, Yousry T, Thornton J.
Neurology 2010; 74: 658-65.

A Copine family member, Cpne8, is a candidate quantitative trait gene for prion disease incubation time in mouse
Lloyd SE, Maytham EG, Grizenkova J, Hummerich H, Collinge J. Neurogenetics 2010; 11: 185-91.

Large-scale immunohistochemical examination for lymphoreticular prion protein in tonsil specimens collected in Britain
de Marco, M. F., Linehan, J., Gill, O. N., Clewley, J. P. and Brandner, S.
  J Path, 10.1002/ 2767

Generation of a panel of antibodies against proteins encoded on human chromosome 21
Wiseman, F., Sheppard, O., Linehan, J., Brandner, S., Tybulewicz, V.L.J., Fisher, E.M.C
Journal of Negative Results in BioMedicine 2010, 9:7

Tau, prions and Ab: the triad of neurodegeneration
Reiniger L, Lukic A, Linehan J, Rudge P, Collinge J, Mead S, Brandner S.
Acta Neuropathol. 2010 May 16.

The Legs at odd angles (Loa) mutation in cytoplasmic dynein ameliorates mitochondrial function in SOD1G93A mouse model for motor neuron disease
Morsi El-Kadi A, Bros-Facer V, Deng W, Philpott A, Stoddart E, Banks G, Jackson GS, Fisher EM, Duchen MR, Greensmith L, Moore AL, Hafezparast M J Biol Chem. 2010 Apr 9

Modification of superoxide dismutase 1 (SOD1) properties by a GFP tag - implications for research into amyotrophic lateral sclerosis (ALS).
Stevens JC,Chia R, Hendriks WT, Bros-Facer V,van Minnen J, Martin JE,Jackson GS,Greensmith L,Schiavo G, Fisher EM 
PLoS One. 2010 Mar 8;5(3):e9541

Genetic Variability in CLU and Its Association with Alzheimer's Disease
Guerreiro RJ, Beck J, Gibbs JR, Santana I, Rossor MN, Schott JM, Nalls MA, Ribeiro H, Santiago B, Fox NC, Oliveira C, Collinge J, Mead S, Singleton A, Hardy J
  PLoS One. 2010 Mar 3;5(3)

Activated BRAF induces gliomas in mice when combined with Ink4a/Arf loss or Akt activation
Robinson JP, VanBrocklin MW, Guilbeault AR, Signorelli DL, Brandner S, Holmen SL.  Oncogene. 2010 Jan 21;29(3):335-44.

Combinations of genetic mutations in the adult neural stem cell compartment determine brain tumour phenotypes
Jacques TS, Swales A, Brzozowski MJ, Henriquez NV, Linehan JM, Mirzadeh Z, O' Malley C, Naumann H, Alvarez-Buylla A, Brandner S.
EMBO J. 2010 Jan 6;29(1):222-35.

Distinct profiles of brain atrophy in frontotemporal lobar degeneration caused by progranulin and tau mutations
Rohrer JD, Ridgway GR, Modat M, Ourselin S, Mead S, Fox NC, Rossor MN, Warren JD Neuroimage 49 2010 Jan 4.

Progressive logopenic/phonological aphasia: Erosion of the language network
Rohrer JD, Ridgway GR, Crutch SJ, Hailstone J, Goll JC, Clarkson MJ, Mead S, Beck J, Mummery C, Ourselin S, Warrington EK, Rossor MN, Warren JD NeuroImage 49 (2010) Jan 1; 984–993.

Variant or sporadic Creutzfeldt-Jakob disease?
Mead S, Hyare H, Collinge J, Rudge P. Lancet 2010; 375: 889; author reply 889-90.

PAW35 Anti-prion protein monoclonal antibodies at low doses effectively treat prion disease in mice without side-effects
Carswell C, Khalili-Shirazi A, Brandner S, Martins S, Drynda R, Collinge J, Mead S, Clarke A.
J Neurol Neurosurg Psychiatry 2010; 81: e33.