Home > Publications Database > Prion disease: a tale of folds and strains. > print

001     136879
005     20240321220142.0
024 7 _ |a 10.1111/bpa.12045
|2 doi
024 7 _ |a pmid:23587138
|2 pmid
024 7 _ |a 1015-6305
|2 ISSN
024 7 _ |a 1750-3639
|2 ISSN
024 7 _ |a altmetric:1397038
|2 altmetric
037 _ _ |a DZNE-2020-03201
041 _ _ |a English
082 _ _ |a 610
100 1 _ |a Kretzschmar, Hans
|0 P:(DE-2719)9000168
|b 0
|e First author
|u dzne
245 _ _ |a Prion disease: a tale of folds and strains.
260 _ _ |a Oxford
|c 2013
|b Wiley-Blackwell
264 _ 1 |3 online
|2 Crossref
|b Wiley
|c 2013-04-16
264 _ 1 |3 print
|2 Crossref
|b Wiley
|c 2013-05-01
336 7 _ |a article
|2 DRIVER
336 7 _ |a Output Types/Journal article
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336 7 _ |a Journal Article
|b journal
|m journal
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|s 1594278197_29750
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336 7 _ |a ARTICLE
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336 7 _ |a JOURNAL_ARTICLE
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336 7 _ |a Journal Article
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520 _ _ |a Research 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.'
536 _ _ |a 342 - Disease Mechanisms and Model Systems (POF3-342)
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|c POF3-342
|f POF III
|x 0
542 _ _ |i 2015-09-01
|2 Crossref
|u http://doi.wiley.com/10.1002/tdm_license_1.1
588 _ _ |a Dataset connected to CrossRef, PubMed,
650 _ 7 |a PrPC Proteins
|2 NLM Chemicals
650 _ 2 |a Animals
|2 MeSH
650 _ 2 |a Blood-Brain Barrier
|2 MeSH
650 _ 2 |a Creutzfeldt-Jakob Syndrome: genetics
|2 MeSH
650 _ 2 |a Creutzfeldt-Jakob Syndrome: pathology
|2 MeSH
650 _ 2 |a Genome-Wide Association Study
|2 MeSH
650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Kuru: genetics
|2 MeSH
650 _ 2 |a Kuru: pathology
|2 MeSH
650 _ 2 |a PrPC Proteins: chemistry
|2 MeSH
650 _ 2 |a PrPC Proteins: genetics
|2 MeSH
650 _ 2 |a PrPC Proteins: toxicity
|2 MeSH
650 _ 2 |a Prion Diseases: genetics
|2 MeSH
650 _ 2 |a Prion Diseases: pathology
|2 MeSH
650 _ 2 |a Prion Diseases: transmission
|2 MeSH
650 _ 2 |a Protein Folding
|2 MeSH
650 _ 2 |a Proteostasis Deficiencies: genetics
|2 MeSH
650 _ 2 |a Proteostasis Deficiencies: pathology
|2 MeSH
700 1 _ |a Tatzelt, Jörg
|0 P:(DE-2719)9000396
|b 1
|e Last author
|u dzne
773 1 8 |a 10.1111/bpa.12045
|b : Wiley, 2013-04-16
|n 3
|p 321-332
|3 journal-article
|2 Crossref
|t Brain Pathology
|v 23
|y 2013
|x 1015-6305
773 _ _ |a 10.1111/bpa.12045
|g Vol. 23, no. 3, p. 321 - 332
|0 PERI:(DE-600)2029927-8
|n 3
|q 23:3<321 - 332
|p 321-332
|t Brain pathology
|v 23
|y 2013
|x 1015-6305
909 C O |o oai:pub.dzne.de:136879
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910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
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910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
|0 I:(DE-588)1065079516
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913 1 _ |a DE-HGF
|b Forschungsbereich Gesundheit
|l Erkrankungen des Nervensystems
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914 1 _ |y 2013
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999 C 5 |a 10.1038/352340a0
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999 C 5 |a 10.1093/hmg/2.5.541
|9 -- missing cx lookup --
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|o 10.1093/hmg/2.5.541
999 C 5 |a 10.1001/archneur.58.11.1899
|9 -- missing cx lookup --
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|o 10.1001/archneur.58.11.1899
999 C 5 |a 10.1038/386232a0
|9 -- missing cx lookup --
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|o 10.1038/386232a0
999 C 5 |a 10.1002/ana.410390613
|9 -- missing cx lookup --
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|o 10.1002/ana.410390613
999 C 5 |a 10.1093/brain/awq234
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999 C 5 |a 10.1073/pnas.95.14.8322
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|o 10.1073/pnas.95.14.8322
999 C 5 |a 10.1007/s00401-012-1002-8
|9 -- missing cx lookup --
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|o 10.1007/s00401-012-1002-8
999 C 5 |a 10.1002/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W
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|o 10.1002/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W
999 C 5 |a 10.1111/j.1750-3639.2008.00187.x
|9 -- missing cx lookup --
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|o 10.1111/j.1750-3639.2008.00187.x
999 C 5 |a 10.1007/s00401-010-0779-6
|9 -- missing cx lookup --
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|o 10.1007/s00401-010-0779-6
999 C 5 |a 10.1007/s00401-009-0585-1
|9 -- missing cx lookup --
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|o 10.1007/s00401-009-0585-1
999 C 5 |a 10.1146/annurev.biochem.69.1.69
|9 -- missing cx lookup --
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|o 10.1146/annurev.biochem.69.1.69
999 C 5 |a 10.1016/S0368-1742(61)80013-1
|9 -- missing cx lookup --
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|o 10.1016/S0368-1742(61)80013-1
999 C 5 |a 10.1110/ps.39201
|9 -- missing cx lookup --
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|o 10.1110/ps.39201
999 C 5 |a 10.1016/S0896-6273(02)00726-2
|9 -- missing cx lookup --
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|o 10.1016/S0896-6273(02)00726-2
999 C 5 |a 10.1073/pnas.182425299
|9 -- missing cx lookup --
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|o 10.1073/pnas.182425299
999 C 5 |a 10.1083/jcb.200304140
|9 -- missing cx lookup --
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|o 10.1083/jcb.200304140
999 C 5 |a 10.1097/00005072-199810000-00010
|9 -- missing cx lookup --
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|o 10.1097/00005072-199810000-00010
999 C 5 |a 10.1093/emboj/20.23.6692
|9 -- missing cx lookup --
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|o 10.1093/emboj/20.23.6692
999 C 5 |a 10.1146/annurev.mi.43.100189.002021
|9 -- missing cx lookup --
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|o 10.1146/annurev.mi.43.100189.002021
999 C 5 |a 10.1126/science.1222951
|9 -- missing cx lookup --
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|o 10.1126/science.1222951
999 C 5 |a 10.1016/0092-8674(84)90533-6
|9 -- missing cx lookup --
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|o 10.1016/0092-8674(84)90533-6
999 C 5 |a 10.1016/0092-8674(90)90134-Z
|9 -- missing cx lookup --
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|o 10.1016/0092-8674(90)90134-Z
999 C 5 |y 1991
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999 C 5 |a 10.1074/jbc.275.25.19121
|9 -- missing cx lookup --
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|o 10.1074/jbc.275.25.19121
999 C 5 |a 10.1091/mbc.E06-01-0083
|9 -- missing cx lookup --
|2 Crossref
|o 10.1091/mbc.E06-01-0083
999 C 5 |a 10.1038/emboj.2008.122
|9 -- missing cx lookup --
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999 C 5 |a 10.1038/sj.emboj.7600462
|9 -- missing cx lookup --
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|o 10.1038/sj.emboj.7600462
999 C 5 |a 10.1038/emboj.2011.86
|9 -- missing cx lookup --
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999 C 5 |a 10.1038/382180a0
|9 -- missing cx lookup --
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999 C 5 |a 10.1016/S0014-5793(97)00920-4
|9 -- missing cx lookup --
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999 C 5 |a 10.1186/1471-2377-10-99
|9 -- missing cx lookup --
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999 C 5 |a 10.1073/pnas.96.23.13044
|9 -- missing cx lookup --
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|o 10.1073/pnas.96.23.13044
999 C 5 |a 10.1038/2654
|9 -- missing cx lookup --
|2 Crossref
|o 10.1038/2654
999 C 5 |a 10.1016/j.neurobiolaging.2011.10.011
|9 -- missing cx lookup --
|2 Crossref
|o 10.1016/j.neurobiolaging.2011.10.011
999 C 5 |a 10.1038/nature09768
|9 -- missing cx lookup --
|2 Crossref
|o 10.1038/nature09768
999 C 5 |a 10.3201/eid1803.110685
|9 -- missing cx lookup --
|2 Crossref
|o 10.3201/eid1803.110685
999 C 5 |a 10.1006/jmbi.1994.0030
|9 -- missing cx lookup --
|2 Crossref
|o 10.1006/jmbi.1994.0030
999 C 5 |a 10.1006/jmbi.2000.5183
|9 -- missing cx lookup --
|2 Crossref
|o 10.1006/jmbi.2000.5183
999 C 5 |a 10.1016/0092-8674(89)90608-9
|9 -- missing cx lookup --
|2 Crossref
|o 10.1016/0092-8674(89)90608-9
999 C 5 |a 10.1073/pnas.94.26.14279
|9 -- missing cx lookup --
|2 Crossref
|o 10.1073/pnas.94.26.14279
999 C 5 |y 2000
|2 Crossref
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999 C 5 |a 10.1073/pnas.96.26.15137
|9 -- missing cx lookup --
|2 Crossref
|o 10.1073/pnas.96.26.15137
999 C 5 |a 10.1002/ana.410430618
|9 -- missing cx lookup --
|2 Crossref
|o 10.1002/ana.410430618
999 C 5 |a 10.1016/S0140-6736(05)78358-6
|9 -- missing cx lookup --
|2 Crossref
|o 10.1016/S0140-6736(05)78358-6
999 C 5 |a 10.1001/archneur.56.3.357
|9 -- missing cx lookup --
|2 Crossref
|o 10.1001/archneur.56.3.357
999 C 5 |a 10.1016/S0092-8674(00)81572-X
|9 -- missing cx lookup --
|2 Crossref
|o 10.1016/S0092-8674(00)81572-X
999 C 5 |a 10.1083/jcb.125.6.1239
|9 -- missing cx lookup --
|2 Crossref
|o 10.1083/jcb.125.6.1239
999 C 5 |a 10.1074/jbc.270.24.14793
|9 -- missing cx lookup --
|2 Crossref
|o 10.1074/jbc.270.24.14793
999 C 5 |a 10.1016/S0014-5793(00)01232-1
|9 -- missing cx lookup --
|2 Crossref
|o 10.1016/S0014-5793(00)01232-1
999 C 5 |a 10.1002/humu.9157
|9 -- missing cx lookup --
|2 Crossref
|o 10.1002/humu.9157
999 C 5 |a 10.1016/0092-8674(87)90150-4
|9 -- missing cx lookup --
|2 Crossref
|o 10.1016/0092-8674(87)90150-4
999 C 5 |a 10.1371/journal.pone.0050623
|9 -- missing cx lookup --
|2 Crossref
|o 10.1371/journal.pone.0050623
999 C 5 |a 10.1523/JNEUROSCI.0105-05.2005
|9 -- missing cx lookup --
|2 Crossref
|o 10.1523/JNEUROSCI.0105-05.2005
999 C 5 |a 10.1021/bi982330q
|9 -- missing cx lookup --
|2 Crossref
|o 10.1021/bi982330q
999 C 5 |a 10.1016/S0014-5793(01)03027-7
|9 -- missing cx lookup --
|2 Crossref
|o 10.1016/S0014-5793(01)03027-7
999 C 5 |a 10.1093/emboj/cdg344
|9 -- missing cx lookup --
|2 Crossref
|o 10.1093/emboj/cdg344
999 C 5 |a 10.1016/S0140-6736(05)62035-1
|9 -- missing cx lookup --
|2 Crossref
|o 10.1016/S0140-6736(05)62035-1
999 C 5 |a 10.4161/pri.6.1.16984
|9 -- missing cx lookup --
|2 Crossref
|o 10.4161/pri.6.1.16984
999 C 5 |a 10.1073/pnas.87.21.8262
|9 -- missing cx lookup --
|2 Crossref
|o 10.1073/pnas.87.21.8262
999 C 5 |a 10.1007/128_2011_166
|9 -- missing cx lookup --
|2 Crossref
|o 10.1007/128_2011_166
999 C 5 |a 10.1126/science.274.5295.2079
|9 -- missing cx lookup --
|2 Crossref
|o 10.1126/science.274.5295.2079
999 C 5 |a 10.1038/nn.3178
|9 -- missing cx lookup --
|2 Crossref
|o 10.1038/nn.3178
999 C 5 |a 10.1093/molbev/msg014
|9 -- missing cx lookup --
|2 Crossref
|o 10.1093/molbev/msg014
999 C 5 |a 10.1111/j.1365-2990.2010.01129.x
|9 -- missing cx lookup --
|2 Crossref
|o 10.1111/j.1365-2990.2010.01129.x
999 C 5 |a 10.1073/pnas.0800190105
|9 -- missing cx lookup --
|2 Crossref
|o 10.1073/pnas.0800190105
999 C 5 |a 10.1073/pnas.77.12.7112
|9 -- missing cx lookup --
|2 Crossref
|o 10.1073/pnas.77.12.7112
999 C 5 |a 10.1038/299691a0
|9 -- missing cx lookup --
|2 Crossref
|o 10.1038/299691a0
999 C 5 |a 10.1016/0092-8674(84)90520-8
|9 -- missing cx lookup --
|2 Crossref
|o 10.1016/0092-8674(84)90520-8
999 C 5 |a 10.1126/science.1183748
|9 -- missing cx lookup --
|2 Crossref
|o 10.1126/science.1183748
999 C 5 |a 10.1016/j.bbadis.2009.02.014
|9 -- missing cx lookup --
|2 Crossref
|o 10.1016/j.bbadis.2009.02.014
999 C 5 |a 10.1016/0092-8674(87)90134-6
|9 -- missing cx lookup --
|2 Crossref
|o 10.1016/0092-8674(87)90134-6
999 C 5 |a 10.1016/S0140-6736(96)91412-9
|9 -- missing cx lookup --
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|o 10.1016/S0140-6736(96)91412-9
999 C 5 |a 10.1099/vir.0.042507-0
|9 -- missing cx lookup --
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|o 10.1099/vir.0.042507-0
999 C 5 |a 10.1007/s004390050204
|9 -- missing cx lookup --
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|o 10.1007/s004390050204
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|9 -- missing cx lookup --
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|o 10.1007/s004390051096
999 C 5 |a 10.1034/j.1600-0854.2003.00088.x
|9 -- missing cx lookup --
|2 Crossref
|o 10.1034/j.1600-0854.2003.00088.x
999 C 5 |a 10.1006/jmbi.1999.2831
|9 -- missing cx lookup --
|2 Crossref
|o 10.1006/jmbi.1999.2831
999 C 5 |a 10.1093/nar/15.7.3191
|9 -- missing cx lookup --
|2 Crossref
|o 10.1093/nar/15.7.3191
999 C 5 |a 10.1111/j.1440-1789.2008.00987.x
|9 -- missing cx lookup --
|2 Crossref
|o 10.1111/j.1440-1789.2008.00987.x
999 C 5 |a 10.1073/pnas.1110789109
|9 -- missing cx lookup --
|2 Crossref
|o 10.1073/pnas.1110789109
999 C 5 |a 10.1002/ana.22094
|9 -- missing cx lookup --
|2 Crossref
|o 10.1002/ana.22094
999 C 5 |a 10.1093/glycob/10.10.959
|9 -- missing cx lookup --
|2 Crossref
|o 10.1093/glycob/10.10.959

LibraryCollectionCLSMajorCLSMinorLanguageAuthor
Marc 21