{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,14]],"date-time":"2026-07-14T03:41:47Z","timestamp":1784000507913,"version":"3.55.0"},"update-to":[{"DOI":"10.1371\/journal.ppat.1012552","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2024,9,23]],"date-time":"2024-09-23T00:00:00Z","timestamp":1727049600000}}],"reference-count":76,"publisher":"Public Library of Science (PLoS)","issue":"9","license":[{"start":{"date-parts":[[2024,9,11]],"date-time":"2024-09-11T00:00:00Z","timestamp":1726012800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100008483","name":"NOMIS Stiftung","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008483","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000781","name":"European Research Council","doi-asserted-by":"publisher","award":["278611"],"award-info":[{"award-number":["278611"]}],"id":[{"id":"10.13039\/501100000781","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100018460","name":"Fondazione Gelu","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100018460","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001711","name":"Schweizerischer Nationalfonds zur F\u00f6rderung der Wissenschaftlichen Forschung","doi-asserted-by":"publisher","award":["179040; 207872; 183563"],"award-info":[{"award-number":["179040; 207872; 183563"]}],"id":[{"id":"10.13039\/501100001711","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004412","name":"Human Frontier Science Program","doi-asserted-by":"publisher","award":["RGP0001\/2022"],"award-info":[{"award-number":["RGP0001\/2022"]}],"id":[{"id":"10.13039\/100004412","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.plospathogens.org"],"crossmark-restriction":false},"short-container-title":["PLoS Pathog"],"abstract":"<jats:p>\n                    In prion diseases (PrDs), aggregates of misfolded prion protein (PrP\n                    <jats:sup>Sc<\/jats:sup>\n                    ) accumulate not only in the brain but also in extraneural organs. This raises the question whether prion-specific pathologies arise also extraneurally. Here we sequenced mRNA transcripts in skeletal muscle, spleen and blood of prion-inoculated mice at eight timepoints during disease progression. We detected gene-expression changes in all three organs, with skeletal muscle showing the most consistent alterations. The glutamate-ammonia ligase (\n                    <jats:italic>GLUL<\/jats:italic>\n                    ) gene exhibited uniform upregulation in skeletal muscles of mice infected with three distinct scrapie prion strains (RML, ME7, and 22L) and in victims of human sporadic Creutzfeldt-Jakob disease.\n                    <jats:italic>GLUL<\/jats:italic>\n                    dysregulation was accompanied by changes in glutamate\/glutamine metabolism, leading to reduced glutamate levels in skeletal muscle. None of these changes were observed in skeletal muscle of humans with amyotrophic lateral sclerosis, Alzheimer\u2019s disease, or dementia with Lewy bodies, suggesting that they are specific to prion diseases. These findings reveal an unexpected metabolic dimension of prion infections and point to a potential role for GLUL dysregulation in the glutamate\/glutamine metabolism in prion-affected skeletal muscle.\n                  <\/jats:p>","DOI":"10.1371\/journal.ppat.1012552","type":"journal-article","created":{"date-parts":[[2024,9,11]],"date-time":"2024-09-11T13:39:25Z","timestamp":1726061965000},"page":"e1012552","update-policy":"https:\/\/doi.org\/10.1371\/journal.ppat.corrections_policy","source":"Crossref","is-referenced-by-count":7,"title":["Prion diseases disrupt glutamate\/glutamine metabolism in skeletal muscle"],"prefix":"10.1371","volume":"20","author":[{"given":"Davide","family":"Caredio","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maru\u0161a","family":"Koderman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karl J.","family":"Frontzek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Sorce","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mario","family":"Nuvolone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juliane","family":"Bremer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanni","family":"Mariutti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petra","family":"Schwarz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lidia","family":"Madrigal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marija","family":"Mitrovic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Sellitto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Streichenberger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Scheckel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0344-6708","authenticated-orcid":true,"given":"Adriano","family":"Aguzzi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2024,9,11]]},"reference":[{"issue":"7","key":"ppat.1012552.ref001","doi-asserted-by":"crossref","first-page":"936","DOI":"10.1038\/nn.3120","article-title":"Prion 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Klein","year":"1997","journal-title":"Nature"},{"issue":"7","key":"ppat.1012552.ref005","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1038\/77401","article-title":"Temporary inactivation of follicular dendritic cells delays neuroinvasion of scrapie","volume":"6","author":"NA Mabbott","year":"2000","journal-title":"Nature medicine"},{"issue":"1","key":"ppat.1012552.ref006","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1186\/2051-5960-1-44","article-title":"Establishing quantitative real-time quaking-induced conversion (qRT-QuIC) for highly sensitive detection and quantification of PrPSc in prion-infected tissues","volume":"1","author":"S Shi","year":"2013","journal-title":"Acta Neuropathologica Communications"},{"issue":"7","key":"ppat.1012552.ref007","doi-asserted-by":"crossref","first-page":"e1008283","DOI":"10.1371\/journal.ppat.1008283","article-title":"Host prion protein expression levels impact prion tropism for the spleen","volume":"16","author":"V 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Houston","year":"2000","journal-title":"Lancet"},{"issue":"2","key":"ppat.1012552.ref011","doi-asserted-by":"crossref","first-page":"e1009276","DOI":"10.1371\/journal.ppat.1009276","article-title":"Preclinical transmission of prions by blood transfusion is influenced by donor genotype and route of infection","volume":"17","author":"MKF Salamat","year":"2021","journal-title":"PLoS pathogens"},{"issue":"1","key":"ppat.1012552.ref012","doi-asserted-by":"crossref","first-page":"34","DOI":"10.3201\/eid2601.181423","article-title":"Preclinical Detection of Prions in Blood of Nonhuman Primates Infected with Variant Creutzfeldt-Jakob Disease","volume":"26","author":"L Concha-Marambio","year":"2020","journal-title":"Emerg Infect Dis"},{"issue":"370","key":"ppat.1012552.ref013","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.aaf6188","article-title":"Detection of prions in blood from patients with variant Creutzfeldt-Jakob disease","volume":"8","author":"L 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