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MRC Prion Unit
From fundamental research to prevention and cure
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2006 Publications

Dissociation of pathological and molecular phenotype of variant Creutzfeldt-Jakob disease in transgenic human prion protein 129 heterozygous mice.

Asante EA, Linehan JM, Gowland I, Joiner S, Fox K, Cooper S, Osiguwa O, Gorry M, Welch J, Houghton R, Desbruslais M, Brandner S, Wadsworth JD, Collinge J. Proc Natl Acad Sci U S A. 2006;103(28):10759-64.

Molecular basis of prion diseases. In: G Siegel, RW Albers, S Brady, D Price, editors. Basic Neurochemistry. 7 ed. 2006
Collinge J, Wadsworth JD. 
p. 791-803.

Kuru in the 21st century--an acquired human prion disease with very long incubation periods.
Collinge J, Whitfield J, McKintosh E, Beck J, Mead S, Thomas DJ, Alpers MP.
Lancet 2006;367(9528):2068-74.

Incubation period of human prion disease - Author's reply
Collinge J, Alpers MP.
Lancet 2006;368(9539):914-5.

A second family with familial AD and the V717L APP mutation has a later age at onset.
Godbolt AK, Beck JA, Collinge J, Cipolotti L, Fox NC, Rossor MN.
Neurology 2006;66:611-2.

Distinct glycoform ratios of protease resistant prion protein associated with PRNP point mutations.
Hill A, Joiner S, Beck J, Campbell TA, Dickinson A, Poulter M, Wadsworth JD, Collinge J.
Brain 2006;129:676-85.

The Human Prion Protein Residue 129 Polymorphism Lies Within a Cluster of Epitopes for T Cell Recognition
Isaacs JD, Ingram RJ, Collinge J, Altmann DM, Jackson GS.
Journal of Neuropathology and Experimental Neurology 2006;65(11):1059-68.

ALS phenotypes with mutations in CHMP2B ( charged multivesiculur body protein 2B)
N. Parkinson PhD, P. G. Ince MD*, M. O. Smith BSc, R. Highley PhD, G. Skibinski PhD, P. M. Andersen MD, K. E. Morrison PhD, H. S. Pall MD,
O. Hardiman PhD, J. Collinge MD, P. J. Shaw MD, E. M.C. Fisher PhD, on behalf of the MRC Proteomics in ALS Study and the FReJA Consortium.
NEUROLOGY 2006;67:1074-1077.

The role of the cellular prion protein in the immune system
Isaacs JD, Jackson GS, Altmann DM.
Clin.Exp.Immunol. 2006;146(1):1-8.

A reassessment of the neuropathy of frontotemporal dementia linked to chromosome 3
Holme, I.E., Englund, E., Mackenzie, I.R.A., Johannsen, P., Isaacs, A.M., Gade, S., Nielsen, J., Gydesen, S., Thursgaard, T., Brown, J.M., Collinge, J., Rossor, M., Fisher, E.M.C. J. Neuropathol. Exp. Neurol. (2007) 66: 884-891.

Novel mutation of the PRNP gene of a clinical CJD case
Kotta K, Paspaltsis I, Bostantjopoulou S, Latsoudis H, Plaitakis A, Kazis D, Collinge J, Sklaviadis T.
BMC Infectious Diseases 2006;6(1):169.

Codon 129 polymorphism of the human prion protein influences the kinetics of amyloid formation
Lewis PA, Tattum MH, Jones S, Bhelt D, Batchelor M, ÿ, Collinge J, Jackson GS
. J.Gen.Virol. 2006;87(Pt 8):2443-9.

Removal of the glycosylphosphatidylinositol anchor from PrP(Sc) by cathepsin D does not reduce prion infectivity
Lewis PA, Properzi F, Prodromidou K, ÿ, Collinge J, Jackson GS.
Biochemical Journal 2006;395(2):443-8.

Inherited prion disease with six octapeptide repeat insertional mutation--molecular analysis of phenotypic heterogeneity
Mead S, Poulter M, Beck J, Webb TE, Campbell TA, Linehan JM, Desbruslais M, Joiner S, Wadsworth JD, King A, Lantos P, Collinge J.

Brain 2006;129(Pt 9):2297-317.

Prion protein (PRNP) genotypes in frontotemporal lobar degeneration syndromes
Rohrer JD, Mead S, Omar R, Poulter M, Warren JD, Collinge J, Rossor MN.
Annals of Neurology 2006;60(5):616.

Elongated oligomers assemble into mammalian PrP amyloid fibrils
Tattum MH, Cohen-Krausz S, Thumanu K, Wharton CW, Khalili-Shirazi A, Jackson GS, Orlova EV, Collinge J, ÿ, Saibil HR.

Journal of Molecular Biology 2006;357(3):975-85.

A systematic review of prion therapeutics in experimental models
Trevitt CR, Collinge J.
Brain 2006;129(Pt 9):2241-65.   Supplemental documents

Molecular Pathology of Prion Diseases. Creutzfeldt-jakob Disease
Wadsworth
J, Collinge J. AABB Press; 2006.

Phenotypic heterogeneity in inherited prion disease (P102L) is associated with differential propagation of protease-resistant wild-type and mutant prion protein
Wadsworth JD, Joiner S, Linehan JM, Cooper S, Powell C, Mallinson G, Buckell J, Gowland I, Asante EA, Budka H, Brandner S, Collinge J.

Brain 2006;129(Pt 6):1557-69.

Update on vCJD prevalence based on tonsil screening. WHO guidelines on tissue infectivity distribution in transmissible spongiform encephalopathies
Wadsworth,J.  P28. 2006.  WHO press, World Health Organisation, Geneva, Switzerland.

Regional brian metabolite abnormalities in inherited prion disease and asymptomatic gene carriers demonstrated in vivo by quantitative proton magnetic resonance spectroscopy.
Waldman AD, Cordery RJ, MacManus DG, Godbolt A, Collinge J, Rossor MN.
Neuroradiology 2006;48(6):428-33.

Multiple forms of copper(II) coordination occur throughout the disordered N-terminal region of the prion protein at pH 7.4
Wells MA, Jelinska C, Hosszu LL, Craven CJ, ÿ, Collinge J, Waltho JP, Jackson GS.
The Biochemical Journal 2006;400(3):501-10.

Familial prion disease in a Hungarian family with a novel 144-base pair insertion in the prion protein gene
T Kovács, J A Beck, M I Papp, P L Lantos, Z Arányi, I G Szirmai, M Farsang, A Stuke, A Csillik, J Collinge.
J Neurol Neurosurg Psychiatry 2007;78:321-323.

A reassessment of copper(II) binding in the full-length prion protein
Wells MA, Jackson GS, Jones S, Hosszu LL, Craven CJ, ÿ, Collinge J, Waltho JP.
  The Biochemical Journal 2006;399(3):435-44.

Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report
Wroe SJ, Pal S, Siddique D, Hyare H, Macfarlane R, Joiner S, Linehan JM, Brandner S, Wadsworth JD, Hewitt P, Collinge J.

Lancet 2006;368(9552):2061-7.