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A novel Gerstmann-Sträussler-Scheinker disease mutation defines a precursor for amyloidogenic 8 kDa PrP fragments and reveals N-terminal structural changes shared by other GSS alleles.
Mercer RCC, Daude N, Dorosh L, Fu ZL, Mays CE, Gapeshina H, Wohlgemuth SL, Acevedo-Morantes CY, Yang J, Cashman NR, Coulthart MB, Pearson DM, Joseph JT, Wille H, Safar JG, Jansen GH, Stepanova M, Sykes BD, Westaway D. Mercer RCC, et al. Among authors: yang j. PLoS Pathog. 2018 Jan 16;14(1):e1006826. doi: 10.1371/journal.ppat.1006826. eCollection 2018 Jan. PLoS Pathog. 2018. PMID: 29338055 Free PMC article.
Down-regulation of Shadoo in prion infections traces a pre-clinical event inversely related to PrP(Sc) accumulation.
Westaway D, Genovesi S, Daude N, Brown R, Lau A, Lee I, Mays CE, Coomaraswamy J, Canine B, Pitstick R, Herbst A, Yang J, Ko KW, Schmitt-Ulms G, Dearmond SJ, McKenzie D, Hood L, Carlson GA. Westaway D, et al. Among authors: yang j. PLoS Pathog. 2011 Nov;7(11):e1002391. doi: 10.1371/journal.ppat.1002391. Epub 2011 Nov 17. PLoS Pathog. 2011. PMID: 22114562 Free PMC article.
Endoproteolytic processing of the mammalian prion glycoprotein family.
Mays CE, Coomaraswamy J, Watts JC, Yang J, Ko KW, Strome B, Mercer RC, Wohlgemuth SL, Schmitt-Ulms G, Westaway D. Mays CE, et al. Among authors: yang j. FEBS J. 2014 Feb;281(3):862-76. doi: 10.1111/febs.12654. Epub 2013 Dec 23. FEBS J. 2014. PMID: 24286250 Free article.
Prion disease tempo determined by host-dependent substrate reduction.
Mays CE, Kim C, Haldiman T, van der Merwe J, Lau A, Yang J, Grams J, Di Bari MA, Nonno R, Telling GC, Kong Q, Langeveld J, McKenzie D, Westaway D, Safar JG. Mays CE, et al. Among authors: yang j. J Clin Invest. 2014 Feb;124(2):847-58. doi: 10.1172/JCI72241. Epub 2014 Jan 16. J Clin Invest. 2014. PMID: 24430187 Free PMC article.
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