{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T21:54:08Z","timestamp":1790891648960,"version":"4.1.0"},"update-to":[{"DOI":"10.1371\/journal.ppat.1014124","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T00:00:00Z","timestamp":1786406400000}}],"reference-count":68,"publisher":"Public Library of Science (PLoS)","issue":"8","license":[{"start":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T00:00:00Z","timestamp":1785801600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000024","name":"Canadian Institutes of Health Research","doi-asserted-by":"publisher","award":["202209PJT"],"award-info":[{"award-number":["202209PJT"]}],"id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"publisher"}]},{"name":"CFI\/ORF","award":["43017"],"award-info":[{"award-number":["43017"]}]},{"DOI":"10.13039\/501100004089","name":"Krembil Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004089","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Krembil Brain Institute","award":["N\/A"],"award-info":[{"award-number":["N\/A"]}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01AI156037"],"award-info":[{"award-number":["R01AI156037"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Canadian Graduate Scholarship"},{"name":"Canadian Graduate Scholarship"},{"name":"Canadian Graduate Scholarship"}],"content-domain":{"domain":["www.plospathogens.org"],"crossmark-restriction":false},"short-container-title":["PLoS Pathog"],"abstract":"<jats:p>\n                    The replacement of a single codon in the human prion gene, causing the substitution of glycine with valine at position 127 (G127V) of the prion protein (PrP), prevents development of prion disease. We set out to explore if prion disease survival extension manifests in mice if the V127 mutant is delivered through a recombinant adeno-associated virus (rAAV) packaged as a self-complementary DNA. The notorious delivery limitations of rAAV vectors were designed to be overcome using a cross-correction approach that relied on the expression of the mutation in the context of glycosylphosphatidylinositoI-anchorless (\u0394GPI) PrP. In this proof-of-concept study, we inoculated Rocky Mountain Laboratory (RML) prions into knock-in mice, in which the endogenous murine prion protein gene (\n                    <jats:italic>Prnp<\/jats:italic>\n                    ) was replaced with the bank vole prion protein gene (Bv\n                    <jats:italic>Prnp<\/jats:italic>\n                    ). Prion-inoculated mice that were retro-orbitally transduced with a protective rAAV vector encoding Bv\n                    <jats:italic>Prnp<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>V127<\/jats:italic>\n                    <\/jats:sup>\n                    \u0394GPI survived ~50 days longer than control mice that were unprotected. A deep proteomic analysis revealed that Bv\n                    <jats:italic>Prnp<\/jats:italic>\n                    <jats:sup>V127<\/jats:sup>\n                    \u0394GPI was protective by slowing perturbations to the proteome observed in late-stage RML prion disease. In addition to capturing details of synaptic decay and depletion of proteins in proximity to PrP, the proteomic dataset revealed the identity of proteins of potential diagnostic value that may be central to the brain\u2019s attempt to fight prion disease by contributing to astrocytosis or microgliosis, by coping with calcium influx, or by enhancing the endoplasmic reticulum processing of essential proteins. Taken together, our results demonstrate that a gene therapy based on a GPI-anchorless PrP containing the G127V mutation can delay the onset of prion disease in mice, providing a framework for development of a corresponding therapy in humans.\n                  <\/jats:p>","DOI":"10.1371\/journal.ppat.1014124","type":"journal-article","created":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T17:39:03Z","timestamp":1785865143000},"page":"e1014124","update-policy":"https:\/\/doi.org\/10.1371\/journal.ppat.corrections_policy","source":"Crossref","is-referenced-by-count":2,"title":["A self-complementary recombinant adeno-associated virus vector coding for an anchorless prion protein carrying the G127V mutation extends survival in a rodent prion disease 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