guest ::
| Home > Publications Database > Substitutions of PrP N-terminal histidine residues modulate scrapie disease pathogenesis and incubation time in transgenic mice. > print |
| Home > Publications Database > Substitutions of PrP N-terminal histidine residues modulate scrapie disease pathogenesis and incubation time in transgenic mice. > print |
| 001 | 139661 | ||
| 005 | 20240321220647.0 | ||
| 024 | 7 | _ | |a 10.1371/journal.pone.0188989 |2 doi |
| 024 | 7 | _ | |a pmid:29220360 |2 pmid |
| 024 | 7 | _ | |a pmc:PMC5722314 |2 pmc |
| 024 | 7 | _ | |a altmetric:30222605 |2 altmetric |
| 037 | _ | _ | |a DZNE-2020-05983 |
| 041 | _ | _ | |a English |
| 082 | _ | _ | |a 610 |
| 100 | 1 | _ | |a Eigenbrod, Sabina |b 0 |
| 245 | _ | _ | |a Substitutions of PrP N-terminal histidine residues modulate scrapie disease pathogenesis and incubation time in transgenic mice. |
| 260 | _ | _ | |a San Francisco, California, US |c 2017 |b PLOS |
| 264 | _ | 1 | |3 online |2 Crossref |b Public Library of Science (PLoS) |c 2017-12-08 |
| 336 | 7 | _ | |a article |2 DRIVER |
| 336 | 7 | _ | |a Output Types/Journal article |2 DataCite |
| 336 | 7 | _ | |a Journal Article |b journal |m journal |0 PUB:(DE-HGF)16 |s 1603715713_24004 |2 PUB:(DE-HGF) |
| 336 | 7 | _ | |a ARTICLE |2 BibTeX |
| 336 | 7 | _ | |a JOURNAL_ARTICLE |2 ORCID |
| 336 | 7 | _ | |a Journal Article |0 0 |2 EndNote |
| 520 | _ | _ | |a Prion diseases have been linked to impaired copper homeostasis and copper induced-oxidative damage to the brain. Divalent metal ions, such as Cu2+ and Zn2+, bind to cellular prion protein (PrPC) at octapeptide repeat (OR) and non-OR sites within the N-terminal half of the protein but information on the impact of such binding on conversion to the misfolded isoform often derives from studies using either OR and non-OR peptides or bacterially-expressed recombinant PrP. Here we created new transgenic mouse lines expressing PrP with disrupted copper binding sites within all four histidine-containing OR's (sites 1-4, H60G, H68G, H76G, H84G, 'TetraH>G' allele) or at site 5 (composed of residues His-95 and His-110; 'H95G' allele) and monitored the formation of misfolded PrP in vivo. Novel transgenic mice expressing PrP(TetraH>G) at levels comparable to wild-type (wt) controls were susceptible to mouse-adapted scrapie strain RML but showed significantly prolonged incubation times. In contrast, amino acid replacement at residue 95 accelerated disease progression in corresponding PrP(H95G) mice. Neuropathological lesions in terminally ill transgenic mice were similar to scrapie-infected wt controls, but less severe. The pattern of PrPSc deposition, however, was not synaptic as seen in wt animals, but instead dense globular plaque-like accumulations of PrPSc in TgPrP(TetraH>G) mice and diffuse PrPSc deposition in (TgPrP(H95G) mice), were observed throughout all brain sections. We conclude that OR and site 5 histidine substitutions have divergent phenotypic impacts and that cis interactions between the OR region and the site 5 region modulate pathogenic outcomes by affecting the PrP globular domain. |
| 536 | _ | _ | |a 344 - Clinical and Health Care Research (POF3-344) |0 G:(DE-HGF)POF3-344 |c POF3-344 |f POF III |x 0 |
| 542 | _ | _ | |i 2017-12-08 |2 Crossref |u http://creativecommons.org/licenses/by/4.0/ |
| 588 | _ | _ | |a Dataset connected to CrossRef, PubMed, |
| 650 | _ | 7 | |a Prion Proteins |2 NLM Chemicals |
| 650 | _ | 7 | |a Histidine |0 4QD397987E |2 NLM Chemicals |
| 650 | _ | 2 | |a Animals |2 MeSH |
| 650 | _ | 2 | |a Histidine: chemistry |2 MeSH |
| 650 | _ | 2 | |a Mice |2 MeSH |
| 650 | _ | 2 | |a Mice, Transgenic |2 MeSH |
| 650 | _ | 2 | |a Prion Proteins: chemistry |2 MeSH |
| 650 | _ | 2 | |a Scrapie: pathology |2 MeSH |
| 700 | 1 | _ | |a Frick, Petra |0 P:(DE-2719)2810661 |b 1 |e Corresponding author |u dzne |
| 700 | 1 | _ | |a Bertsch, Uwe |b 2 |
| 700 | 1 | _ | |a Mitteregger-Kretzschmar, Gerda |b 3 |
| 700 | 1 | _ | |a Mielke, Janina |b 4 |
| 700 | 1 | _ | |a Maringer, Marko |b 5 |
| 700 | 1 | _ | |a Piening, Niklas |b 6 |
| 700 | 1 | _ | |a Hepp, Alexander |b 7 |
| 700 | 1 | _ | |a Daude, Nathalie |b 8 |
| 700 | 1 | _ | |a Windl, Otto |0 P:(DE-HGF)0 |b 9 |
| 700 | 1 | _ | |a Levin, Johannes |0 P:(DE-HGF)0 |b 10 |
| 700 | 1 | _ | |a Giese, Armin |0 P:(DE-HGF)0 |b 11 |
| 700 | 1 | _ | |a Sakthivelu, Vignesh |0 P:(DE-HGF)0 |b 12 |
| 700 | 1 | _ | |a Tatzelt, Jörg |0 P:(DE-HGF)0 |b 13 |
| 700 | 1 | _ | |a Kretzschmar, Hans |0 P:(DE-HGF)0 |b 14 |
| 700 | 1 | _ | |a Westaway, David |b 15 |
| 773 | 1 | 8 | |a 10.1371/journal.pone.0188989 |b : Public Library of Science (PLoS), 2017-12-08 |n 12 |p e0188989 |3 journal-article |2 Crossref |t PLOS ONE |v 12 |y 2017 |x 1932-6203 |
| 773 | _ | _ | |a 10.1371/journal.pone.0188989 |g Vol. 12, no. 12, p. e0188989 - |0 PERI:(DE-600)2267670-3 |n 12 |q 12:12 |t PLOS ONE |v 12 |y 2017 |x 1932-6203 |
| 856 | 4 | _ | |y OpenAccess |u https://pub.dzne.de/record/139661/files/journal.pone.0188989.pdf |
| 856 | 4 | _ | |y OpenAccess |x icon |u https://pub.dzne.de/record/139661/files/journal.pone.0188989.gif?subformat=icon |
| 856 | 4 | _ | |y OpenAccess |x icon-1440 |u https://pub.dzne.de/record/139661/files/journal.pone.0188989.jpg?subformat=icon-1440 |
| 856 | 4 | _ | |y OpenAccess |x icon-180 |u https://pub.dzne.de/record/139661/files/journal.pone.0188989.jpg?subformat=icon-180 |
| 856 | 4 | _ | |y OpenAccess |x icon-640 |u https://pub.dzne.de/record/139661/files/journal.pone.0188989.jpg?subformat=icon-640 |
| 856 | 4 | _ | |y OpenAccess |x pdfa |u https://pub.dzne.de/record/139661/files/journal.pone.0188989.pdf?subformat=pdfa |
| 856 | 7 | _ | |2 Pubmed Central |u http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5722314 |
| 909 | C | O | |o oai:pub.dzne.de:139661 |p openaire |p open_access |p VDB |p driver |p dnbdelivery |
| 910 | 1 | _ | |a Deutsches Zentrum für Neurodegenerative Erkrankungen |0 I:(DE-588)1065079516 |k DZNE |b 1 |6 P:(DE-2719)2810661 |
| 913 | 1 | _ | |a DE-HGF |b Forschungsbereich Gesundheit |l Erkrankungen des Nervensystems |1 G:(DE-HGF)POF3-340 |0 G:(DE-HGF)POF3-344 |2 G:(DE-HGF)POF3-300 |v Clinical and Health Care Research |x 0 |
| 914 | 1 | _ | |y 2017 |
| 915 | _ | _ | |a Creative Commons Attribution CC BY (No Version) |0 LIC:(DE-HGF)CCBYNV |2 V:(DE-HGF) |b DOAJ |
| 915 | _ | _ | |a OpenAccess |0 StatID:(DE-HGF)0510 |2 StatID |
| 915 | _ | _ | |a JCR |0 StatID:(DE-HGF)0100 |2 StatID |b PLOS ONE : 2021 |d 2022-11-16 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0200 |2 StatID |b SCOPUS |d 2022-11-16 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0300 |2 StatID |b Medline |d 2022-11-16 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0501 |2 StatID |b DOAJ Seal |d 2022-04-12T10:14:32Z |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0500 |2 StatID |b DOAJ |d 2022-04-12T10:14:32Z |
| 915 | _ | _ | |a Peer Review |0 StatID:(DE-HGF)0030 |2 StatID |b DOAJ : Blind peer review |d 2022-04-12T10:14:32Z |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0600 |2 StatID |b Ebsco Academic Search |d 2022-11-16 |
| 915 | _ | _ | |a Peer Review |0 StatID:(DE-HGF)0030 |2 StatID |b ASC |d 2022-11-16 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0199 |2 StatID |b Clarivate Analytics Master Journal List |d 2022-11-16 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)0150 |2 StatID |b Web of Science Core Collection |d 2022-11-16 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)1050 |2 StatID |b BIOSIS Previews |d 2022-11-16 |
| 915 | _ | _ | |a DBCoverage |0 StatID:(DE-HGF)1040 |2 StatID |b Zoological Record |d 2022-11-16 |
| 915 | _ | _ | |a IF < 5 |0 StatID:(DE-HGF)9900 |2 StatID |d 2022-11-16 |
| 920 | 1 | _ | |0 I:(DE-2719)1210003 |k AG Neumann |l Molecular Neuropathology of Neurodegenerative Diseases |x 0 |
| 980 | _ | _ | |a journal |
| 980 | _ | _ | |a VDB |
| 980 | _ | _ | |a UNRESTRICTED |
| 980 | _ | _ | |a I:(DE-2719)1210003 |
| 980 | 1 | _ | |a FullTexts |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1073/pnas.95.23.13363 |2 Crossref |o 10.1073/pnas.95.23.13363 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/382180a0 |2 Crossref |o 10.1038/382180a0 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1093/emboj/18.12.3193 |2 Crossref |o 10.1093/emboj/18.12.3193 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/370471a0 |2 Crossref |o 10.1038/370471a0 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1002/j.1460-2075.1996.tb00467.x |2 Crossref |o 10.1002/j.1460-2075.1996.tb00467.x |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/S0896-6273(00)00046-5 |2 Crossref |o 10.1016/S0896-6273(00)00046-5 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1110/ps.051434005 |2 Crossref |o 10.1110/ps.051434005 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1021/bi027138+ |2 Crossref |o 10.1021/bi027138+ |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1074/jbc.M708472200 |2 Crossref |o 10.1074/jbc.M708472200 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1021/bi050251q |2 Crossref |o 10.1021/bi050251q |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.brainres.2003.09.014 |2 Crossref |o 10.1016/j.brainres.2003.09.014 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1073/pnas.0903807106 |2 Crossref |o 10.1073/pnas.0903807106 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1074/jbc.C300303200 |2 Crossref |o 10.1074/jbc.C300303200 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s00775-009-0564-y |2 Crossref |o 10.1007/s00775-009-0564-y |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1006/bbrc.2000.2270 |2 Crossref |o 10.1006/bbrc.2000.2270 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1074/jbc.M007243200 |2 Crossref |o 10.1074/jbc.M007243200 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.biolcel.2003.11.007 |2 Crossref |o 10.1016/j.biolcel.2003.11.007 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1074/jbc.M507327200 |2 Crossref |o 10.1074/jbc.M507327200 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1529/biophysj.108.139568 |2 Crossref |o 10.1529/biophysj.108.139568 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1073/pnas.121190898 |2 Crossref |o 10.1073/pnas.121190898 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.jsb.2006.06.013 |2 Crossref |o 10.1016/j.jsb.2006.06.013 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1080/15287390903084389 |2 Crossref |o 10.1080/15287390903084389 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/S0960-9822(01)00147-6 |2 Crossref |o 10.1016/S0960-9822(01)00147-6 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/0169-328X(95)00175-R |2 Crossref |o 10.1016/0169-328X(95)00175-R |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1021/ja053254z |2 Crossref |o 10.1021/ja053254z |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.15252/emmm.201404588 |2 Crossref |o 10.15252/emmm.201404588 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/S1050-3862(96)00167-2 |2 Crossref |o 10.1016/S1050-3862(96)00167-2 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/356577a0 |2 Crossref |o 10.1038/356577a0 |
| 999 | C | 5 | |y 1986 |2 Crossref |o GA Carlson 1986 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1002/bies.950050612 |2 Crossref |o 10.1002/bies.950050612 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/0021-9975(68)90006-6 |2 Crossref |o 10.1016/0021-9975(68)90006-6 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1099/0022-1317-83-3-695 |2 Crossref |o 10.1099/0022-1317-83-3-695 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/2161310a0 |2 Crossref |o 10.1038/2161310a0 |
| 999 | C | 5 | |y 1996 |2 Crossref |o ME Bruce 1996 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/S0002-9440(10)64705-0 |2 Crossref |o 10.1016/S0002-9440(10)64705-0 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1074/jbc.M405652200 |2 Crossref |o 10.1074/jbc.M405652200 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/S0140-6736(63)90393-3 |2 Crossref |o 10.1016/S0140-6736(63)90393-3 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1074/jbc.M512239200 |2 Crossref |o 10.1074/jbc.M512239200 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/S0076-6879(99)09011-4 |2 Crossref |o 10.1016/S0076-6879(99)09011-4 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1128/JVI.78.20.11051-11060.2004 |2 Crossref |o 10.1128/JVI.78.20.11051-11060.2004 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1002/j.1460-2075.1986.tb04539.x |2 Crossref |o 10.1002/j.1460-2075.1986.tb04539.x |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.bbrc.2004.06.075 |2 Crossref |o 10.1016/j.bbrc.2004.06.075 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/BF00266934 |2 Crossref |o 10.1007/BF00266934 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1097/00005072-198505000-00006 |2 Crossref |o 10.1097/00005072-198505000-00006 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1515/bchm.1998.379.11.1307 |2 Crossref |o 10.1515/bchm.1998.379.11.1307 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1515/BC.2001.081 |2 Crossref |o 10.1515/BC.2001.081 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1046/j.1471-4159.2001.00625.x |2 Crossref |o 10.1046/j.1471-4159.2001.00625.x |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1046/j.1471-4159.2001.00522.x |2 Crossref |o 10.1046/j.1471-4159.2001.00522.x |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1042/bj3620253 |2 Crossref |o 10.1042/bj3620253 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1074/jbc.M510606200 |2 Crossref |o 10.1074/jbc.M510606200 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1021/bi800253c |2 Crossref |o 10.1021/bi800253c |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1136/jnnp.2003.020198 |2 Crossref |o 10.1136/jnnp.2003.020198 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1073/pnas.88.23.10926 |2 Crossref |o 10.1073/pnas.88.23.10926 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1371/journal.ppat.1000390 |2 Crossref |o 10.1371/journal.ppat.1000390 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1074/jbc.M506521200 |2 Crossref |o 10.1074/jbc.M506521200 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1073/pnas.0610827104 |2 Crossref |o 10.1073/pnas.0610827104 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1007/s12031-010-9387-0 |2 Crossref |o 10.1007/s12031-010-9387-0 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1126/sciadv.1600014 |2 Crossref |o 10.1126/sciadv.1600014 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1111/j.1750-3639.2007.00061.x |2 Crossref |o 10.1111/j.1750-3639.2007.00061.x |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.freeradbiomed.2008.08.024 |2 Crossref |o 10.1016/j.freeradbiomed.2008.08.024 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1111/j.1742-4658.2008.06680.x |2 Crossref |o 10.1111/j.1742-4658.2008.06680.x |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.mcn.2005.07.001 |2 Crossref |o 10.1016/j.mcn.2005.07.001 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1074/jbc.273.50.33107 |2 Crossref |o 10.1074/jbc.273.50.33107 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/0014-5793(96)01104-0 |2 Crossref |o 10.1016/0014-5793(96)01104-0 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1039/c0mt00037j |2 Crossref |o 10.1039/c0mt00037j |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/0092-8674(87)90134-6 |2 Crossref |o 10.1016/0092-8674(87)90134-6 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/ng0298-118 |2 Crossref |o 10.1038/ng0298-118 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1529/biophysj.106.083642 |2 Crossref |o 10.1529/biophysj.106.083642 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/srep15253 |2 Crossref |o 10.1038/srep15253 |
| 999 | C | 5 | |y 2010 |2 Crossref |o IH Solomon 2010 |
| 999 | C | 5 | |y 2004 |2 Crossref |o Q Kong 2004 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1073/pnas.151038498 |2 Crossref |o 10.1073/pnas.151038498 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.2174/138920309789352056 |2 Crossref |o 10.2174/138920309789352056 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1074/jbc.M113.502351 |2 Crossref |o 10.1074/jbc.M113.502351 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.str.2012.12.002 |2 Crossref |o 10.1016/j.str.2012.12.002 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1016/j.str.2016.04.017 |2 Crossref |o 10.1016/j.str.2016.04.017 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1074/jbc.M111.265645 |2 Crossref |o 10.1074/jbc.M111.265645 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1074/jbc.M110.145516 |2 Crossref |o 10.1074/jbc.M110.145516 |
| 999 | C | 5 | |9 -- missing cx lookup -- |a 10.1038/nature07761 |2 Crossref |o 10.1038/nature07761 |
| Library | Collection | CLSMajor | CLSMinor | Language | Author |
|---|