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MRC Prion Unit
From fundamental research to prevention and cure
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Latest research findings from the Unit

Early microgliosis precedes neuronal loss and behavioural impairment in mice with a frontotemporal dementia-causing CHMP2B mutation. 
Clayton EL, Mancuso R, Nielsen TT, Mizielinska S, Holmes H, Powell N, Norona F, Larsen JO, Milioto C, Wilson KM, Lythgoe MF, Ourselin S, Nielsen JE, Johannsen P, Holm I, Collinge J; FReJA., Oliver PL, Gomez-Nicola D, Isaacs AM. Hum Mol Genet. 2017 Jan 16. doi: 10.1093/hmg/ddx003. [Epub ahead of print]

Cultural factors that affected the spatial and temporal epidemiology of kuru. 
Whitfield J. T., Pako, W. H., Collinge, J. & Alpers, M. P. (2017) R Soc open sci
DOI:

Neuroanatomical correlates of prion disease progression - a 3T longitudinal voxel-based morphometry study. 
De Vita E, Ridgway GR, White MJ, Porter MC, Caine D, Rudge P, Collinge J, Yousry TA, Jager HR, Mead S, Thornton JS, Hyare H.  Neuroimage Clin. 2016 Nov 2;13:89-96. eCollection 2017.
 

Mammalian prions and their wider relevance in neurodegenerative diseases 
Collinge J. Nature. 2016 Nov 10; 539(7628):217-226. doi: 10.1038/nature20415.

Genetic architecture of sporadic frontotemporal dementia and overlap with Alzheimer's and Parkinson's diseases. 
Ferrari R, Wang Y, Vandrovcova J, Guelfi S, Witeolar A, Karch CM, Schork AJ, Fan CC, Brewer JB; International FTD-Genomics Consortium (IFGC); International Parkinson's Disease Genomics Consortium (IPDGC),; International Genomics of Alzheimer's Project (IGAP),, Momeni P, Schellenberg GD, Dillon WP, Sugrue LP, Hess CP, Yokoyama JS, Bonham LW, Rabinovici GD, Miller BL, Andreassen OA, Dale AM, Hardy J, Desikan RS. . J Neurol Neurosurg Psychiatry. 2017 Feb; 88(2):152-164. doi: 10.1136/jnnp-2016-314411. Epub 2016 Nov 29.

Pathologically confirmed variant CJD in a prion protein gene codon 129 heterozygous patient. 
Tze How Mok, Zane Jaunmuktane, Susan Joiner, Tracy Campbell, Catherine Morgan, Benjamin Wakerley, Farhad Golestani, Peter Rudge, Simon Mead, Hans Rolf Jäger,  Jonathan D F Wadsworth, Sebastian Brandner, John Collinge  NEJM In Press Nov 2016

Structural Variation in Amyloid-β Fibrils from Alzheimer's Disease Clinical Subtypes 
Qiang W, Yau W-M, Lu J-X, Collinge J & Tycko R. Nature. Nov 2016 in press doi:10.1038/nature20814

Diagnosing Sporadic Creutzfeldt-Jakob Disease by the Detection of Abnormal Prion Protein in Patient Urine. 
Luk C, Jones S, Thomas C, Fox NC, Mok TH, Mead S, Collinge J, Jackson GS  JAMA Neurol. 2016; 3. doi: 10.1001/jamaneurol.2016.3733. [Epub ahead of print]

Collinge et al. reply
Collinge J, Jaunmuktane Z, Mead S, Rudge P, Brandner S.   Nature 2016;535(7611):E2-3. 

Quantitative EEG parameters correlate with the progression of human prion diseases. 
Franko E, Wehner T, Joly O, Lowe J, Porter MC, Kenny J, Thompson A, Rudge P, Collinge J, Mead S.   J Neurol Neurosurg Psychiatry 2016; 313501; doi:10.1136/jnnp-2016-313501

Physical, chemical and kinetic factors affecting prion infectivity.  
Properzi F, Badhan A, Klier S, Schmidt C, Klöhn PC, Wadsworth JDF, Clarke AR, Jackson GS, Collinge J.  Prion 2016; doi: 10.1080/19336896.2016.1181250 

Ex vivo mammalian prions are formed of paired double helical prion protein fibrils.   
Terry
 CWenborn AGros NSells JJoiner SHosszu LLPTattum HPanico S Clare DKCollinge JSaibil HRWadsworth JDF. Open Biol. 2016: 160035; http://dx.doi.org/10.1098/rsob.160035

Variants of PLCXD3 are not associated with variant or sporadic Creutzfeldt-Jakob disease in a large international study. 
Balendra R, Uphill J, Collinson C, Druyeh R, Adamson G, Hummerich H, Zerr I, Gambetti P, Collinge J, Mead S. BMC Med Genet. 2016; 17(1):28. doi: 10.1186/s12881-016-0278-2   

Genetic risk factors for the posterior cortical atrophy variant of Alzheimer’s disease. 
Schott JM, Crutch SJ, Carrasquillo MM, Uphill J, Shakespeare TJ, Ryan NS, Yong KX, Lehmann M, Ertekin-Taner N, Graff-Radford NR, Boeve BF, Murray ME, Khan QU, Petersen RC, Dickson DW, Knopman DS, Rabinovici GD, Miller BL, Gonzalez AS, Gil-Néciga E, Snowden JS, Harris J, Pickering-Brown SM, Louwersheimer E, van der Flier WM, Scheltens P, Pijnenburg YA, Galasko D, Sarazin M, Dubois B, Magnin E, Galimberti D, Scarpini E, Cappa SF, Hodges JR, Halliday GM, Bartley L, Carrillo MC, Bras JT, Hardy J, Rossor MN, Collinge J, Fox NC, Mead S. Alzheimers Dement. 2016; 12(8):862-71. doi: 10.1016/j.jalz.2016.01.010

Clinical Trial Simulations Based on Genetic Stratification and the Natural History of a Functional Outcome Measure in Creutzfeldt-Jakob Disease.
Mead S, Burnell M, Lowe J, Thompson A, Lukic A, Porter MC, Carswell C, Kaski D, Kenny J, Mok TH, Bjurstrom N, Franko E, Gorham M, Druyeh R, Wadsworth JD, Jaunmuktane Z, Brandner S, Hyare H, Rudge P, Walker AS, Collinge J. JAMA Neurol. 2016; 73(4):447-55. doi: 10.1001/jamaneurol.2015.4885

Prion-mediated neurodegeneration is associated with early impairment of the ubiquitin-proteasome system. 
McKinnon C, Goold R, Andre R, Devoy A, Ortega Z, Moonga J, Linehan JM, Brandner S, Lucas JJ, Collinge J, Tabrizi SJ. Acta Neuropathol. 2016; 131(3):411-25. doi: 10.1007/s00401-015-1508-y.

MRC Prion News