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000139661 0247_ $$2doi$$a10.1371/journal.pone.0188989
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000139661 037__ $$aDZNE-2020-05983
000139661 041__ $$aEnglish
000139661 082__ $$a610
000139661 1001_ $$aEigenbrod, Sabina$$b0
000139661 245__ $$aSubstitutions of PrP N-terminal histidine residues modulate scrapie disease pathogenesis and incubation time in transgenic mice.
000139661 260__ $$aSan Francisco, California, US$$bPLOS$$c2017
000139661 264_1 $$2Crossref$$3online$$bPublic Library of Science (PLoS)$$c2017-12-08
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000139661 520__ $$aPrion 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.
000139661 536__ $$0G:(DE-HGF)POF3-344$$a344 - Clinical and Health Care Research (POF3-344)$$cPOF3-344$$fPOF III$$x0
000139661 542__ $$2Crossref$$i2017-12-08$$uhttp://creativecommons.org/licenses/by/4.0/
000139661 588__ $$aDataset connected to CrossRef, PubMed,
000139661 650_7 $$2NLM Chemicals$$aPrion Proteins
000139661 650_7 $$04QD397987E$$2NLM Chemicals$$aHistidine
000139661 650_2 $$2MeSH$$aAnimals
000139661 650_2 $$2MeSH$$aHistidine: chemistry
000139661 650_2 $$2MeSH$$aMice
000139661 650_2 $$2MeSH$$aMice, Transgenic
000139661 650_2 $$2MeSH$$aPrion Proteins: chemistry
000139661 650_2 $$2MeSH$$aScrapie: pathology
000139661 7001_ $$0P:(DE-2719)2810661$$aFrick, Petra$$b1$$eCorresponding author$$udzne
000139661 7001_ $$aBertsch, Uwe$$b2
000139661 7001_ $$aMitteregger-Kretzschmar, Gerda$$b3
000139661 7001_ $$aMielke, Janina$$b4
000139661 7001_ $$aMaringer, Marko$$b5
000139661 7001_ $$aPiening, Niklas$$b6
000139661 7001_ $$aHepp, Alexander$$b7
000139661 7001_ $$aDaude, Nathalie$$b8
000139661 7001_ $$0P:(DE-HGF)0$$aWindl, Otto$$b9
000139661 7001_ $$0P:(DE-HGF)0$$aLevin, Johannes$$b10
000139661 7001_ $$0P:(DE-HGF)0$$aGiese, Armin$$b11
000139661 7001_ $$0P:(DE-HGF)0$$aSakthivelu, Vignesh$$b12
000139661 7001_ $$0P:(DE-HGF)0$$aTatzelt, Jörg$$b13
000139661 7001_ $$0P:(DE-HGF)0$$aKretzschmar, Hans$$b14
000139661 7001_ $$aWestaway, David$$b15
000139661 77318 $$2Crossref$$3journal-article$$a10.1371/journal.pone.0188989$$b : Public Library of Science (PLoS), 2017-12-08$$n12$$pe0188989$$tPLOS ONE$$v12$$x1932-6203$$y2017
000139661 773__ $$0PERI:(DE-600)2267670-3$$a10.1371/journal.pone.0188989$$gVol. 12, no. 12, p. e0188989 -$$n12$$pe0188989$$q12:12<e0188989 -$$tPLOS ONE$$v12$$x1932-6203$$y2017
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000139661 8567_ $$2Pubmed Central$$uhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC5722314
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000139661 9131_ $$0G:(DE-HGF)POF3-344$$1G:(DE-HGF)POF3-340$$2G:(DE-HGF)POF3-300$$aDE-HGF$$bForschungsbereich Gesundheit$$lErkrankungen des Nervensystems$$vClinical and Health Care Research$$x0
000139661 9141_ $$y2017
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