000088416 001__ 88416
000088416 005__ 20210902121642.0
000088416 0247_ $$2doi$$a10.1038/s41598-020-61977-1
000088416 0248_ $$2sideral$$a117112
000088416 037__ $$aART-2020-117112
000088416 041__ $$aeng
000088416 100__ $$0(orcid)0000-0002-7037-6316$$aBarrio, T.
000088416 245__ $$aMixtures of prion substrains in natural scrapie cases revealed by ovinised murine models
000088416 260__ $$c2020
000088416 5060_ $$aAccess copy available to the general public$$fUnrestricted
000088416 5203_ $$aPhenotypic variability in prion diseases, such as scrapie, is associated to the existence of prion strains, which are different pathogenic prion protein (PrPSc) conformations with distinct pathobiological properties. To faithfully study scrapie strain variability in natural sheep isolates, transgenic mice expressing sheep cellular prion protein (PrPC) are used. In this study, we used two of such models to bioassay 20 scrapie isolates from the Spain-France-Andorra transboundary territory. Animals were intracerebrally inoculated and survival periods, proteinase K-resistant PrP (PrPres) banding patterns, lesion profiles and PrPSc distribution were studied. Inocula showed a remarkable homogeneity on banding patterns, all of them but one showing 19-kDa PrPres. However, a number of isolates caused accumulation of 21-kDa PrPres in TgShp XI. A different subgroup of isolates caused long survival periods and presence of 21-kDa PrPres in Tg338 mice. It seemed that one major 19-kDa prion isoform and two distinct 21-kDa variants coexisted in source inocula, and that they could be separated by bioassay in each transgenic model. The reason why each model favours a specific component of the mixture is unknown, although PrPC expression level may play a role. Our results indicate that coinfection with more than one substrain is more frequent than infection with a single component.
000088416 536__ $$9info:eu-repo/grantAgreement/ES/MINECO/AGL2015-65560-R$$9info:eu-repo/grantAgreement/ES/MEC/FPU14-04348$$9info:eu-repo/grantAgreement/EUR/INTERREG-POCTEFA/EFA-148-16 REDPRION
000088416 540__ $$9info:eu-repo/semantics/openAccess$$aby$$uhttp://creativecommons.org/licenses/by/3.0/es/
000088416 590__ $$a4.379$$b2020
000088416 591__ $$aMULTIDISCIPLINARY SCIENCES$$b17 / 73 = 0.233$$c2020$$dQ1$$eT1
000088416 592__ $$a1.24$$b2020
000088416 593__ $$aMultidisciplinary$$c2020$$dQ1
000088416 655_4 $$ainfo:eu-repo/semantics/article$$vinfo:eu-repo/semantics/publishedVersion
000088416 700__ $$0(orcid)0000-0003-3352-740X$$aFilali, H.$$uUniversidad de Zaragoza
000088416 700__ $$0(orcid)0000-0001-9075-2764$$aOtero, A.$$uUniversidad de Zaragoza
000088416 700__ $$aSheleby-Elías, J.
000088416 700__ $$0(orcid)0000-0002-1590-3347$$aMarín, B.$$uUniversidad de Zaragoza
000088416 700__ $$aVidal, E.
000088416 700__ $$aBéringue, V.
000088416 700__ $$aTorres, J.M.
000088416 700__ $$aGroschup, M.
000088416 700__ $$aAndréoletti, O.
000088416 700__ $$0(orcid)0000-0002-7173-7216$$aBadiola, J.J.$$uUniversidad de Zaragoza
000088416 700__ $$0(orcid)0000-0002-2746-3932$$aBolea, R.$$uUniversidad de Zaragoza
000088416 7102_ $$11009$$2773$$aUniversidad de Zaragoza$$bDpto. Patología Animal$$cÁrea Sanidad Animal
000088416 773__ $$g10 (2020), 5042  [15 pp.]$$pSci. rep.$$tScientific Reports$$x2045-2322
000088416 8564_ $$s4419939$$uhttps://zaguan.unizar.es/record/88416/files/texto_completo.pdf$$yVersión publicada
000088416 8564_ $$s203113$$uhttps://zaguan.unizar.es/record/88416/files/texto_completo.jpg?subformat=icon$$xicon$$yVersión publicada
000088416 909CO $$ooai:zaguan.unizar.es:88416$$particulos$$pdriver
000088416 951__ $$a2021-09-02-09:01:01
000088416 980__ $$aARTICLE