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dc.contributor.authorManson, Jean C.en
dc.contributor.authorJamieson, Elizabethen
dc.contributor.authorBaybutt, Herberten
dc.contributor.authorTuzi, Nadia L.en
dc.contributor.authorBarron, Ronaen
dc.contributor.authorMcConnell, Ireneen
dc.contributor.authorSomerville, Roberten
dc.contributor.authorIronside, Jamesen
dc.contributor.authorWill, Roberten
dc.contributor.authorSy, Man-Sunen
dc.contributor.authorMelton, David W.en
dc.contributor.authorHope, Jamesen
dc.contributor.authorBostock, Christopheren
dc.identifier.citationManson, J.C. (1999) ‘A single amino acid alteration (101l) introduced into murine PrP dramatically alters incubation time of transmissible spongiform encephalopathy’, The EMBO Journal, 18(23), pp. 6855–6864. Available at:
dc.descriptionRona Barron - ORCID: 0000-0003-4512-9177
dc.descriptionItem is not available in this repository.en
dc.description.abstractA mutation equivalent to P102L in the human PrP gene, associated with Gerstmann–Straussler syndrome (GSS), has been introduced into the murine PrP gene by gene targeting. Mice homozygous for this mutation (101LL) showed no spontaneous transmissible spongiform encephalopathy (TSE) disease, but had incubation times dramatically different from wild-type mice following inoculation with different TSE sources. Inoculation with GSS produced disease in 101LL mice in 288 days. Disease was transmitted from these mice to both wild-type (226 days) and 101LL mice (148 days). In contrast, 101LL mice infected with ME7 had prolonged incubation times (338 days) compared with wild-type mice (161 days). The 101L mutation does not, therefore, produce any spontaneous genetic disease in mice but significantly alters the incubation time of TSE infection. Additionally, a rapid TSE transmission was demonstrated despite extremely low levels of disease-associated PrP.en
dc.publisherEMBO Pressen
dc.relation.ispartofThe EMBO Journalen
dc.titleA single amino acid alteration (101L) introduced into murine PrP dramatically alters incubation time of transmissible spongiform encephalopathyen
rioxxterms.typeJournal Article/Reviewen

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