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MRC Prion Unit
From fundamental research to prevention and cure
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2008 Publications

Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin
Cronier S, Gros N, Tattum MH, Jackson GS, Clarke AR, Collinge J, Wadsworth JD. Biochem J. 2008 Dec 1;416(2):297-305.

Successful amplification of degraded DNA for use with high-throughput SNP genotyping platforms
Mead S, Poulter M, Beck J, Uphill J, Jones C, Ang CE, Mein CA, Collinge J. Hum Mutat. 2008 Dec;29(12):1452-8.

The end of kuru: 50 years of research into an extraordinary disease
Collinge J, Alpers MP. Philos Trans R Soc Lond B Biol Sci. 2008 Nov 27;363(1510):3689-96.

The epidemiology of kuru: monitoring the epidemic from its peak to its end
Alpers MP. Philos Trans R Soc Lond B Biol Sci. 2008 Nov 27;363(1510):3707-13.

Real-time quantitative PCR analysis of pediatric ependymomas identifies novelcandidate genes including TPR at 1q25 and CHIBBY at 22q12-q13
Karakoula K, Suarez-Merino B, Ward S, Phipps KP, Harkness W, Hayward R, Thompson D, Jacques TS, Harding B, Beck J, Thomas DG, Warr TJ. Genes Chromosomes Cancer 2008 Nov; 47 (11):1005-22.

Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease
Brandner S, Whitfield J, Boone K, Puwa A, O'Malley C, Linehan JM, Joiner S, Scaravilli F, Calder I, P Alpers M, Wadsworth JD, Collinge J.Philos Trans R Soc Lond B Biol Sci. 2008 Nov 27;363(1510):3755-63.

Review. The origin of the prion agent of kuru: molecular and biological strain typing
Wadsworth JD, Joiner S, Linehan JM, Asante EA, Brandner S, Collinge J. Philos Trans R Soc Lond B Biol Sci. 2008 Nov 27;363(1510):3747-53.

Genetic susceptibility, evolution and the kuru epidemic
Mead S, Whitfield J, Poulter M, Shah P, Uphill J, Beck J, Campbell T, Al-Dujaily H, Hummerich H, Alpers MP, Collinge J.
Philos Trans R Soc Lond B Biol Sci. 2008 Nov 27;363(1510):3741-6

A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea
Collinge J, Whitfield J, McKintosh E, Frosh A, Mead S, Hill AF, Brandner S, Thomas D, Alpers MP.
Philos Trans R Soc Lond B Biol Sci. 2008 Nov 27;363(1510):3725-39.

Mortuary rites of the South Fore and kuru
Whitfield JT, Pako WH, Collinge J, Alpers MP. Philos Trans R Soc Lond B Biol Sci. 2008 Nov 27;363(1510):3721-4.

Review. Lessons of kuru research: background to recent studies with some personal reflections
Collinge J. Philos Trans R Soc Lond B Biol Sci. 2008 Nov 27;363(1510):3689-96.

Reminiscences and reflections on kuru, personal and scientific.
Collinge J, Alpers MP.Philos Trans R Soc Lond B Biol Sci. 2008 Nov 27;363(1510):3613.

Introduction
Collinge J, Alpers MP. Philos Trans R Soc Lond B Biol Sci. 2008 Nov 27;363(1510):3607-12.

Association of a null allele of SPRN with variant Creutzfeldt-Jakob disease
Beck JA, Campbell TA, Adamson G, Poulter M, Uphill JB, Molou E, Collinge J, Mead S. J Med Genet. 2008 Dec;45(12):813-7. Epub 2008 Sep 19.

Phenotypic heterogeneity and genetic modification of P102L inherited prion disease in an international series
Webb TE, Poulter M, Beck J, Uphill J, Adamson G, Campbell T, Linehan J, Powell C, Brandner S, Pal S, Siddique D, Wadsworth JD, Joiner S, Alner K, Petersen C, Hampson S, Rhymes C, Treacy C, Storey E, Geschwind MD, Nemeth AH, Wroe S, Collinge J, Mead S.
Brain. 2008 Oct;131(Pt 10):2632-46. Epub 2008 Aug 30.

Hippocampal bursts caused by changes in NMDA receptor-dependent excitation in a mouse model of variant CJD
Ratté S, Prescott SA, Collinge J, Jefferys JG. Neurobiol Dis. 2008 Oct;32(1):96-104. Epub 2008 Jun 26

Investigation of mcp1 as a quantitative trait gene for prion disease incubation time in mouse
O'Shea M, Maytham EG, Linehan JM, Brandner S, Collinge J, Lloyd SE. Genetics. 2008 Sep;180(1):559-66. Epub 2008 Aug 20.

First report of Creutzfeldt-Jakob disease occurring in 2 siblings unexplained by PRNP mutation
Webb TE, Pal S, Siddique D, Heaney DC, Linehan JM, Wadsworth JD, Joiner S, Beck J, Wroe SJ, Stevenson V, Brandner S, Mead S, Collinge J.J Neuropathol Exp Neurol. 2008 Sep;67(9):838-41.

Huntington's disease phenocopies are clinically and genetically heterogeneous
Wild EJ, Mudanohwo EE, Sweeney MG, Schnieder SA, Beck J, Bhatia KP, Rossor MN, Davis MB, Tabrizi SJ. Mov Disord 2008; 23: 716-20.

Age of onset and death in inherited prion disease are heritable.
Webb TE, Whittaker J, Collinge J, Mead S. Am J Med Genet B Neuropsychiatr Genet. 2008 Aug 26.

Mapping the progression of progranulin-associated frontotemporal lobar degeneration.
Rohrer JD, Warren JD, Barnes J, Mead S, Beck J, Pepple T, Boyes R, Omar R, Collinge J, Stevens JM, Warrington EK, Rossor MN, Fox NC.
Nat Clin Pract Neurol. 2008 Aug;4(8):455-60. Epub 2008 Jul 22.

Lack of TAR-DNA binding protein-43 (TDP-43) pathology in human prion diseases.
Isaacs AM, Powell C, Webb TE, Linehan JM, Collinge J, Brandner S. Neuropathol Appl Neurobiol. 2008 Aug;34(4):446-56.

Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease.
White MD, Farmer M, Mirabile I, Brandner S, Collinge J, Mallucci GR. Proc Natl Acad Sci U S A. 2008 Jul 22;105(29):10238-43. Epub 2008 Jul 16.

 Frontotemporal dementia linked to chromosome 3 (FTD-3)--current concepts and the detection of a previously unknown branch of the Danish FTD-3 family.
Lindquist SG, Braedgaard H, Svenstrup K, Isaacs AM, Nielsen JE; FReJA Consortium. Eur J Neurol. 2008 Jul;15(7):667-70. Epub 2008 May 15.

Assaying Prions in Cell Culture: The Standard Scrapie Cell Assay (SSCA) and the Scrapie Cell Assay in End Point Format (SCEPA)
Mahal SP, Demczyk CA, Smith EW, Jr., Klohn PC, Weissmann C. Methods Mol Biol 2008; 459: 49-68

The cellular prion protein is preferentially expressed by CD4(+) CD25(+) Foxp3(+) regulatory T cells.
Isaacs JD, Garden OA, Kaur G, Collinge J, Jackson GS, Altmann DM. Immunology. 2008 May 6.

Alterations in Ca2+-buffering in prion-null mice: association with reduced afterhyperpolarizations in CA1 hippocampal neurons.
Powell AD, Toescu EC, Collinge J, Jefferys JG. J Neurosci. 2008 Apr 9;28(15):3877-86.

Parietal lobe deficits in frontotemporal lobar degeneration caused by a mutation in the progranulin gene
Rohrer JD, Warren JD, Omar R, Mead S, Beck J, Revesz T, Holton J, Stevens JM, Al-Sarraj S, Pickering-Brown SM, Hardy J, Fox NC, Collinge J, Warrington EK, Rossor MN. Arch Neurol. 2008 Apr;65(4):506-13.

Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice
Wadsworth JD, Joiner S, Linehan JM, Desbruslais M, Fox K, Cooper S, Cronier S, Asante EA, Mead S, Brandner S, Hill AF, Collinge J.
Proc Natl Acad Sci U S A. 2008 Mar 11;105(10):3885-90. Epub 2008 Mar 3.

A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a large UK series.
Beck J, Rohrer JD, Campbell T, Isaacs A, Morrison KE, Goodall EF, Warrington EK, Stevens J, Revesz T, Holton J, Al-Sarraj S, King A, Scahill R, Warren JD, Fox NC, Rossor MN, Collinge J, Mead S. Brain. 2008 Mar;131(Pt 3):706-20. Epub 2008 Jan 29.

CHMP2B C-truncating mutations in frontotemporal lobar degeneration are associated with an aberrant endosomal phenotype in vitro.
van der Zee J, Urwin H, Engelborghs S, Bruyland M, Vandenberghe R, Dermaut B, De Pooter T, Peeters K, Santens P, De Deyn PP, Fisher EM, Collinge J, Isaacs AM, Van Broeckhoven C. Hum Mol Genet. 2008 Jan 15;17(2):313-22. Epub 2007 Oct 22.

Molecular diagnosis of human prion disease.
Wadsworth JD, Powell C, Beck JA, Joiner S, Linehan JM, Brandner S, Mead S, Collinge J. Methods Mol Biol. 2008;459:197-227.