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Biol"},{"issue":"26","key":"ref68","doi-asserted-by":"crossref","first-page":"20213","DOI":"10.1074\/jbc.M109.093310","article-title":"Conservation of a glycine-rich region in the prion protein is required for uptake of prion infectivity","volume":"285","author":"CF Harrison","year":"2010","journal-title":"J Biol Chem"},{"key":"ref69","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1016\/S0378-1119(03)00707-8","article-title":"Shadoo, a new protein highly conserved from fish to mammals and with similarity to prion protein","volume":"314","author":"M Premzl","year":"2003","journal-title":"Gene"},{"issue":"17","key":"ref70","doi-asserted-by":"crossref","first-page":"4038","DOI":"10.1038\/sj.emboj.7601830","article-title":"The CNS glycoprotein Shadoo has PrP(C)-like protective properties and displays reduced levels in prion infections","volume":"26","author":"JC Watts","year":"2007","journal-title":"Embo J"},{"key":"ref71","article-title":"Wild-type Shadoo proteins convert to amyloid-like forms under native conditions","author":"N Daude","year":"2010","journal-title":"J Neurochem"},{"issue":"5","key":"ref72","doi-asserted-by":"crossref","first-page":"2690","DOI":"10.1128\/JVI.02720-13","article-title":"Pathogenic mutations within the hydrophobic domain of the prion protein lead to the formation of protease-sensitive prion species with increased lethality","volume":"88","author":"BM Coleman","year":"2014","journal-title":"J Virol"},{"issue":"1","key":"ref73","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1111\/j.1432-1033.1982.tb05919.x","article-title":"pH and temperature dependences of thermolysin catalysis. Catalytic role of zinc-coordinated water","volume":"124","author":"S Kunugi","year":"1982","journal-title":"Eur J Biochem"},{"issue":"3","key":"ref74","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1016\/j.jmb.2003.09.048","article-title":"The octapeptide repeats in mammalian prion protein constitute a pH-dependent folding and aggregation site","volume":"334","author":"R Zahn","year":"2003","journal-title":"J Mol Biol"},{"key":"ref75","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1006\/bbrc.1993.1447","article-title":"An amber mutation of prion protein in Gerstmann-Str\u00e4ussler syndrome with mutant PrP plaques","volume":"192","author":"T Kitamoto","year":"1993","journal-title":"Biochem Biophys Res Commun"},{"issue":"1","key":"ref76","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1016\/j.cell.2005.01.034","article-title":"Fibril conformation as the basis of species- and strain-dependent seeding specificity of mammalian prion amyloids","volume":"121","author":"EM Jones","year":"2005","journal-title":"Cell"},{"issue":"48","key":"ref77","doi-asserted-by":"crossref","first-page":"13851","DOI":"10.1073\/pnas.1610716113","article-title":"Amyloid fibrils from the N-terminal prion protein fragment are infectious","volume":"113","author":"JK Choi","year":"2016","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"7335","key":"ref78","doi-asserted-by":"crossref","first-page":"540","DOI":"10.1038\/nature09768","article-title":"Prion propagation and toxicity in vivo occur in two distinct mechanistic phases","volume":"470","author":"MK Sandberg","year":"2011","journal-title":"Nature"},{"key":"ref79","doi-asserted-by":"crossref","first-page":"9811","DOI":"10.1073\/pnas.85.24.9811","article-title":"Nerve growth factor increases mRNA levels for the prion protein and the beta-amyloid protein precursor in developing hamster brain","volume":"85","author":"WC Mobley","year":"1988","journal-title":"Proc Natl Acad Sci USA"},{"key":"ref80","first-page":"394","article-title":"Unconventional Virus Diseases of the Central Nervous System","author":"MP McKinley","year":"1989"},{"key":"ref81","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1242\/dev.115.1.117","article-title":"The prion protein gene: a role in mouse embryogenesis?","volume":"115","author":"J Manson","year":"1992","journal-title":"Development"},{"key":"ref82","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1016\/j.brainres.2006.12.055","article-title":"Developmental expression of PrP in the preimplantation embryo","volume":"1139","author":"P Tremblay","year":"2007","journal-title":"Brain Research"},{"issue":"6","key":"ref83","doi-asserted-by":"crossref","first-page":"724","DOI":"10.1038\/nbt969","article-title":"Time-controlled transcardiac perfusion cross-linking for the study of protein interactions in complex tissues","volume":"22","author":"G Schmitt-Ulms","year":"2004","journal-title":"Nat Biotechnol"},{"issue":"52","key":"ref84","doi-asserted-by":"crossref","first-page":"37241","DOI":"10.1074\/jbc.M113.488650","article-title":"The prion protein modulates A-type K+ currents mediated by Kv4.2 complexes through dipeptidyl aminopeptidase-like protein 6","volume":"288","author":"RC Mercer","year":"2013","journal-title":"J Biol Chem"},{"issue":"3","key":"ref85","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1016\/S0896-6273(02)01185-6","article-title":"The CD26-related dipeptidyl aminopeptidase-like protein DPPX is a critical component of neuronal A-type K+ channels","volume":"37","author":"MS Nadal","year":"2003","journal-title":"Neuron"},{"key":"ref86","doi-asserted-by":"crossref","first-page":"2270","DOI":"10.1038\/ncomms3270","article-title":"DPP6 regulation of dendritic morphogenesis impacts hippocampal synaptic development","volume":"4","author":"L Lin","year":"2013","journal-title":"Nat Commun"},{"issue":"4","key":"ref87","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1007\/s10048-014-0418-9","article-title":"DPP6 gene disruption in a family with Gilles de la Tourette syndrome","volume":"15","author":"P Prontera","year":"2014","journal-title":"Neurogenetics"},{"key":"ref88","article-title":"Implication of LRRC4C and DPP6 in neurodevelopmental disorders","author":"G Maussion","year":"2016","journal-title":"Am J Med Genet A"},{"key":"ref89","doi-asserted-by":"crossref","first-page":"767","DOI":"10.1002\/ana.410390613","article-title":"Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease","volume":"39","author":"P Parchi","year":"1996","journal-title":"Ann Neurol"},{"key":"ref90","doi-asserted-by":"crossref","first-page":"10168","DOI":"10.1073\/pnas.97.18.10168","article-title":"Genetic influence on the structural variations of the abnormal prion protein","volume":"97","author":"P Parchi","year":"2000","journal-title":"PNAS"},{"issue":"9","key":"ref91","doi-asserted-by":"crossref","first-page":"3501","DOI":"10.1073\/pnas.0409651102","article-title":"Diagnosis of human prion disease","volume":"102","author":"JG Safar","year":"2005","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref92","article-title":"Coexistence of Distinct Prion Types Enables Conformational Evolution of Human PrPSc by Competitive Selection","author":"T Haldiman","year":"2013","journal-title":"J Biol Chem"},{"issue":"7","key":"ref93","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1016\/S1474-4422(12)70063-7","article-title":"Sporadic human prion diseases: molecular insights and diagnosis","volume":"11","author":"G Puoti","year":"2012","journal-title":"Lancet neurology"},{"issue":"9","key":"ref94","doi-asserted-by":"crossref","first-page":"e1002242","DOI":"10.1371\/journal.ppat.1002242","article-title":"Protease-sensitive conformers in broad spectrum of distinct PrPSc structures in sporadic Creutzfeldt-Jakob disease are indicator of progression rate","volume":"7","author":"C Kim","year":"2011","journal-title":"PLoS Pathog"},{"issue":"8","key":"ref95","doi-asserted-by":"crossref","first-page":"e1002835","DOI":"10.1371\/journal.ppat.1002835","article-title":"Small protease sensitive oligomers of PrPSc in distinct human prions determine conversion rate of PrP(C)","volume":"8","author":"C Kim","year":"2012","journal-title":"PLoS Pathog"},{"key":"ref96","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1093\/bmb\/66.1.213","article-title":"Sporadic and familial CJD: classification and characterisation","volume":"66","author":"P Gambetti","year":"2003","journal-title":"Br Med Bull"},{"issue":"19","key":"ref97","doi-asserted-by":"crossref","first-page":"14083","DOI":"10.1074\/jbc.C110.113464","article-title":"Mammalian prions generated from bacterially expressed prion protein in the absence of any mammalian cofactors","volume":"285","author":"JI Kim","year":"2010","journal-title":"J Biol Chem"},{"issue":"Pt 10","key":"ref98","doi-asserted-by":"crossref","first-page":"2643","DOI":"10.1093\/brain\/awp196","article-title":"Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics","volume":"132","author":"I Cali","year":"2009","journal-title":"Brain"},{"issue":"4","key":"ref99","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1007\/s00401-012-1002-8","article-title":"Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA","volume":"124","author":"P Parchi","year":"2012","journal-title":"Acta Neuropathol"},{"key":"ref100","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1016\/S1050-3862(96)00167-2","article-title":"A vector for expressing foreign genes in the brains and hearts of transgenic mice","volume":"13","author":"DR Borchelt","year":"1996","journal-title":"Genetic Analysis"},{"issue":"24","key":"ref101","doi-asserted-by":"crossref","first-page":"21562","DOI":"10.1074\/jbc.M100710200","article-title":"Early-onset amyloid deposition and cognitive deficits in transgenic mice expressing a double mutant form of amyloid precursor protein 695","volume":"276","author":"MA Chishti","year":"2001","journal-title":"J Biol Chem"},{"issue":"4","key":"ref102","doi-asserted-by":"crossref","first-page":"1365","DOI":"10.2353\/ajpath.2006.051250","article-title":"Cortical neuronal and glial pathology in TgTauP301L transgenic mice: neuronal degeneration, memory disturbance, and phenotypic variation","volume":"169","author":"T Murakami","year":"2006","journal-title":"Am J Pathol"},{"issue":"3","key":"ref103","doi-asserted-by":"crossref","first-page":"339","DOI":"10.15252\/emmm.201404588","article-title":"Octarepeat region flexibility impacts prion function, endoproteolysis and disease manifestation","volume":"7","author":"A Lau","year":"2015","journal-title":"EMBO Mol Med"},{"issue":"1","key":"ref104","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1073\/pnas.97.1.145","article-title":"NMR solution structure of the human prion protein","volume":"97","author":"R Zahn","year":"2000","journal-title":"Proc Natl Acad Sci USA"},{"issue":"10","key":"ref105","doi-asserted-by":"crossref","first-page":"2172","DOI":"10.1002\/pro.231","article-title":"Differential stability of the bovine prion protein upon urea unfolding","volume":"18","author":"O Julien","year":"2009","journal-title":"Protein Sci"},{"issue":"2","key":"ref106","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1093\/bioinformatics\/bti770","article-title":"The SWISS-MODEL workspace: a web-based environment for protein structure homology modelling","volume":"22","author":"K Arnold","year":"2006","journal-title":"Bioinformatics"},{"issue":"17","key":"ref107","doi-asserted-by":"crossref","first-page":"3389","DOI":"10.1093\/nar\/25.17.3389","article-title":"Gapped BLAST and PSI-BLAST: a new generation of protein database search programs","volume":"25","author":"SF Altschul","year":"1997","journal-title":"Nucleic Acids Res"},{"issue":"2","key":"ref108","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1038\/nmeth.1818","article-title":"HHblits: lightning-fast iterative protein sequence searching by HMM-HMM alignment","volume":"9","author":"M Remmert","year":"2012","journal-title":"Nature methods"},{"issue":"3","key":"ref109","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1093\/bioinformatics\/btq662","article-title":"Toward the estimation of the absolute quality of individual protein structure models","volume":"27","author":"P Benkert","year":"2011","journal-title":"Bioinformatics"},{"issue":"7","key":"ref110","doi-asserted-by":"crossref","first-page":"2284","DOI":"10.1021\/ct200133y","article-title":"Improved Treatment of Ligands and Coupling Effects in Empirical Calculation and Rationalization of pKa Values","volume":"7","author":"CR Sondergaard","year":"2011","journal-title":"J Chem Theory Comput"},{"key":"ref111","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/0010-4655(95)00042-E","article-title":"GROMACS: A message-passing parallel molecular dynamics implementation","volume":"91","author":"HJC Berendsen","year":"1995","journal-title":"Comput Phys Commun"},{"issue":"45","key":"ref112","doi-asserted-by":"crossref","first-page":"11225","DOI":"10.1021\/ja9621760","article-title":"Development and testing of the OPLS all-atom force field on conformational energetics and properties of organic liquids","volume":"118","author":"WL Jorgensen","year":"1996","journal-title":"J Am Chem Soc"},{"issue":"1\u20133","key":"ref113","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/0010-4655(95)00042-E","article-title":"GROMACS: a message-passing parallel molecular dynamics implementation","volume":"91","author":"HJ Berendsen","year":"1995","journal-title":"Computer Physics Communications"},{"issue":"1","key":"ref114","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/0263-7855(96)00018-5","article-title":"VMD: visual molecular dynamics","volume":"14","author":"W Humphrey","year":"1996","journal-title":"J Mol Graph"},{"key":"ref115","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/j.compbiolchem.2014.11.007","article-title":"Tri-peptide reference structures for the calculation of relative solvent accessible surface area in protein amino acid residues","volume":"54","author":"CM Topham","year":"2015","journal-title":"Computational biology and chemistry"},{"key":"ref116","first-page":"btt260","article-title":"InterEvScore: a novel coarse-grained interface scoring function using a multi-body statistical potential coupled to evolution","author":"J Andreani","year":"2013","journal-title":"Bioinformatics"},{"issue":"5","key":"ref117","doi-asserted-by":"crossref","first-page":"051918","DOI":"10.1103\/PhysRevE.76.051918","article-title":"Dynamics of essential collective motions in proteins: theory","volume":"76","author":"M Stepanova","year":"2007","journal-title":"Physical Review E"},{"issue":"7","key":"ref118","doi-asserted-by":"crossref","first-page":"1488","DOI":"10.1021\/bi802043h","article-title":"Structural domains and main-chain flexibility in prion proteins","volume":"48","author":"N Blinov","year":"2009","journal-title":"Biochemistry (Mosc)"},{"issue":"2","key":"ref119","doi-asserted-by":"crossref","first-page":"020901","DOI":"10.1103\/PhysRevE.85.020901","article-title":"Conformational modes in biomolecules: Dynamics and approximate invariance","volume":"85","author":"A Potapov","year":"2012","journal-title":"Physical Review E"},{"issue":"7","key":"ref120","doi-asserted-by":"crossref","first-page":"1847","DOI":"10.1002\/prot.24082","article-title":"Exploring the essential collective dynamics of interacting proteins: Application to prion protein dimers","volume":"80","author":"BB Issack","year":"2012","journal-title":"Proteins: Structure, Function, and Bioinformatics"},{"issue":"22","key":"ref121","doi-asserted-by":"crossref","first-page":"3005","DOI":"10.1093\/bioinformatics\/btp493","article-title":"Mobyle: a new full web bioinformatics framework","volume":"25","author":"B Neron","year":"2009","journal-title":"Bioinformatics"}],"updated-by":[{"DOI":"10.1371\/journal.ppat.1006826","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2018,1,26]],"date-time":"2018-01-26T00:00:00Z","timestamp":1516924800000}}],"container-title":["PLOS 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