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000136879 0247_ $$2doi$$a10.1111/bpa.12045
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000136879 1001_ $$0P:(DE-2719)9000168$$aKretzschmar, Hans$$b0$$eFirst author$$udzne
000136879 245__ $$aPrion disease: a tale of folds and strains.
000136879 260__ $$aOxford$$bWiley-Blackwell$$c2013
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000136879 520__ $$aResearch on prions, the infectious agents of devastating neurological diseases in humans and animals, has been in the forefront of developing the concept of protein aggregation diseases. Prion diseases are distinguished from other neurodegenerative diseases by three peculiarities. First, prion diseases, in addition to being sporadic or genetic like all other neurodegenerative diseases, are infectious diseases. Animal models were developed early on (a long time before the advent of transgenic technology), and this has made possible the discovery of the prion protein as the infectious agent. Second, human prion diseases have true equivalents in animals, such as scrapie, which has been the subject of experimental research for many years. Variant Creutzfeldt-Jakob disease (vCJD) is a zoonosis caused by bovine spongiform encephalopathy (BSE) prions. Third, they show a wide variety of phenotypes in humans and animals, much wider than the variants of any other sporadic or genetic neurodegenerative disease. It has now become firmly established that particular PrP(Sc) isoforms are closely related to specific human prion strains. The variety of human prion diseases, still an enigma in its own right, is a focus of this article. Recently, a series of experiments has shown that the concept of aberrant protein folding and templating, first developed for prions, may apply to a variety of neurodegenerative diseases. In the wake of these discoveries, the term prion has come to be used for Aβ, α-synuclein, tau and possibly others. The self-propagation of alternative conformations seems to be the common denominator of these 'prions,' which in future, in order to avoid confusion, may have to be specified either as 'neurodegenerative prions' or 'infectious prions.'
000136879 536__ $$0G:(DE-HGF)POF3-342$$a342 - Disease Mechanisms and Model Systems (POF3-342)$$cPOF3-342$$fPOF III$$x0
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000136879 650_7 $$2NLM Chemicals$$aPrPC Proteins
000136879 650_2 $$2MeSH$$aAnimals
000136879 650_2 $$2MeSH$$aBlood-Brain Barrier
000136879 650_2 $$2MeSH$$aCreutzfeldt-Jakob Syndrome: genetics
000136879 650_2 $$2MeSH$$aCreutzfeldt-Jakob Syndrome: pathology
000136879 650_2 $$2MeSH$$aGenome-Wide Association Study
000136879 650_2 $$2MeSH$$aHumans
000136879 650_2 $$2MeSH$$aKuru: genetics
000136879 650_2 $$2MeSH$$aKuru: pathology
000136879 650_2 $$2MeSH$$aPrPC Proteins: chemistry
000136879 650_2 $$2MeSH$$aPrPC Proteins: genetics
000136879 650_2 $$2MeSH$$aPrPC Proteins: toxicity
000136879 650_2 $$2MeSH$$aPrion Diseases: genetics
000136879 650_2 $$2MeSH$$aPrion Diseases: pathology
000136879 650_2 $$2MeSH$$aPrion Diseases: transmission
000136879 650_2 $$2MeSH$$aProtein Folding
000136879 650_2 $$2MeSH$$aProteostasis Deficiencies: genetics
000136879 650_2 $$2MeSH$$aProteostasis Deficiencies: pathology
000136879 7001_ $$0P:(DE-2719)9000396$$aTatzelt, Jörg$$b1$$eLast author$$udzne
000136879 77318 $$2Crossref$$3journal-article$$a10.1111/bpa.12045$$b : Wiley, 2013-04-16$$n3$$p321-332$$tBrain Pathology$$v23$$x1015-6305$$y2013
000136879 773__ $$0PERI:(DE-600)2029927-8$$a10.1111/bpa.12045$$gVol. 23, no. 3, p. 321 - 332$$n3$$p321-332$$q23:3<321 - 332$$tBrain pathology$$v23$$x1015-6305$$y2013
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000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1073/pnas.95.14.8322$$o10.1073/pnas.95.14.8322
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1007/s00401-012-1002-8$$o10.1007/s00401-012-1002-8
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1002/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W$$o10.1002/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1111/j.1750-3639.2008.00187.x$$o10.1111/j.1750-3639.2008.00187.x
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1007/s00401-010-0779-6$$o10.1007/s00401-010-0779-6
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1007/s00401-009-0585-1$$o10.1007/s00401-009-0585-1
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1146/annurev.biochem.69.1.69$$o10.1146/annurev.biochem.69.1.69
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/S0368-1742(61)80013-1$$o10.1016/S0368-1742(61)80013-1
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1110/ps.39201$$o10.1110/ps.39201
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/S0896-6273(02)00726-2$$o10.1016/S0896-6273(02)00726-2
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1073/pnas.182425299$$o10.1073/pnas.182425299
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1083/jcb.200304140$$o10.1083/jcb.200304140
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1097/00005072-199810000-00010$$o10.1097/00005072-199810000-00010
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1093/emboj/20.23.6692$$o10.1093/emboj/20.23.6692
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1146/annurev.mi.43.100189.002021$$o10.1146/annurev.mi.43.100189.002021
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1126/science.1222951$$o10.1126/science.1222951
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/0092-8674(84)90533-6$$o10.1016/0092-8674(84)90533-6
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/0092-8674(90)90134-Z$$o10.1016/0092-8674(90)90134-Z
000136879 999C5 $$2Crossref$$oPuckett 1991$$y1991
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1074/jbc.275.25.19121$$o10.1074/jbc.275.25.19121
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1091/mbc.E06-01-0083$$o10.1091/mbc.E06-01-0083
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1038/emboj.2008.122$$o10.1038/emboj.2008.122
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1038/sj.emboj.7600462$$o10.1038/sj.emboj.7600462
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1038/emboj.2011.86$$o10.1038/emboj.2011.86
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1038/382180a0$$o10.1038/382180a0
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/S0014-5793(97)00920-4$$o10.1016/S0014-5793(97)00920-4
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1186/1471-2377-10-99$$o10.1186/1471-2377-10-99
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1073/pnas.96.23.13044$$o10.1073/pnas.96.23.13044
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1038/2654$$o10.1038/2654
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/j.neurobiolaging.2011.10.011$$o10.1016/j.neurobiolaging.2011.10.011
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1038/nature09768$$o10.1038/nature09768
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.3201/eid1803.110685$$o10.3201/eid1803.110685
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1006/jmbi.1994.0030$$o10.1006/jmbi.1994.0030
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1006/jmbi.2000.5183$$o10.1006/jmbi.2000.5183
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/0092-8674(89)90608-9$$o10.1016/0092-8674(89)90608-9
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1073/pnas.94.26.14279$$o10.1073/pnas.94.26.14279
000136879 999C5 $$2Crossref$$oScott 2000$$y2000
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1073/pnas.96.26.15137$$o10.1073/pnas.96.26.15137
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1002/ana.410430618$$o10.1002/ana.410430618
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/S0140-6736(05)78358-6$$o10.1016/S0140-6736(05)78358-6
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1001/archneur.56.3.357$$o10.1001/archneur.56.3.357
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/S0092-8674(00)81572-X$$o10.1016/S0092-8674(00)81572-X
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1083/jcb.125.6.1239$$o10.1083/jcb.125.6.1239
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1074/jbc.270.24.14793$$o10.1074/jbc.270.24.14793
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/S0014-5793(00)01232-1$$o10.1016/S0014-5793(00)01232-1
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1002/humu.9157$$o10.1002/humu.9157
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/0092-8674(87)90150-4$$o10.1016/0092-8674(87)90150-4
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1371/journal.pone.0050623$$o10.1371/journal.pone.0050623
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1523/JNEUROSCI.0105-05.2005$$o10.1523/JNEUROSCI.0105-05.2005
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1021/bi982330q$$o10.1021/bi982330q
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/S0014-5793(01)03027-7$$o10.1016/S0014-5793(01)03027-7
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1093/emboj/cdg344$$o10.1093/emboj/cdg344
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/S0140-6736(05)62035-1$$o10.1016/S0140-6736(05)62035-1
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.4161/pri.6.1.16984$$o10.4161/pri.6.1.16984
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1073/pnas.87.21.8262$$o10.1073/pnas.87.21.8262
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1007/128_2011_166$$o10.1007/128_2011_166
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1126/science.274.5295.2079$$o10.1126/science.274.5295.2079
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1038/nn.3178$$o10.1038/nn.3178
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1093/molbev/msg014$$o10.1093/molbev/msg014
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1111/j.1365-2990.2010.01129.x$$o10.1111/j.1365-2990.2010.01129.x
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1073/pnas.0800190105$$o10.1073/pnas.0800190105
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1073/pnas.77.12.7112$$o10.1073/pnas.77.12.7112
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1038/299691a0$$o10.1038/299691a0
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/0092-8674(84)90520-8$$o10.1016/0092-8674(84)90520-8
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1126/science.1183748$$o10.1126/science.1183748
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/j.bbadis.2009.02.014$$o10.1016/j.bbadis.2009.02.014
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/0092-8674(87)90134-6$$o10.1016/0092-8674(87)90134-6
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1016/S0140-6736(96)91412-9$$o10.1016/S0140-6736(96)91412-9
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1099/vir.0.042507-0$$o10.1099/vir.0.042507-0
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1007/s004390050204$$o10.1007/s004390050204
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1007/s004390051096$$o10.1007/s004390051096
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1034/j.1600-0854.2003.00088.x$$o10.1034/j.1600-0854.2003.00088.x
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1006/jmbi.1999.2831$$o10.1006/jmbi.1999.2831
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1093/nar/15.7.3191$$o10.1093/nar/15.7.3191
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1111/j.1440-1789.2008.00987.x$$o10.1111/j.1440-1789.2008.00987.x
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1073/pnas.1110789109$$o10.1073/pnas.1110789109
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1002/ana.22094$$o10.1002/ana.22094
000136879 999C5 $$2Crossref$$9-- missing cx lookup --$$a10.1093/glycob/10.10.959$$o10.1093/glycob/10.10.959