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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: safar jg. 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.
Branched polyamines cure prion-infected neuroblastoma cells.
Supattapone S, Wille H, Uyechi L, Safar J, Tremblay P, Szoka FC, Cohen FE, Prusiner SB, Scott MR. Supattapone S, et al. J Virol. 2001 Apr;75(7):3453-61. doi: 10.1128/JVI.75.7.3453-3461.2001. J Virol. 2001. PMID: 11238871 Free PMC 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: safar jg. 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.
Octarepeat region flexibility impacts prion function, endoproteolysis and disease manifestation.
Lau A, McDonald A, Daude N, Mays CE, Walter ED, Aglietti R, Mercer RC, Wohlgemuth S, van der Merwe J, Yang J, Gapeshina H, Kim C, Grams J, Shi B, Wille H, Balachandran A, Schmitt-Ulms G, Safar JG, Millhauser GL, Westaway D. Lau A, et al. Among authors: safar jg. EMBO Mol Med. 2015 Mar;7(3):339-56. doi: 10.15252/emmm.201404588. EMBO Mol Med. 2015. PMID: 25661904 Free PMC article.
118 results