{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T20:03:48Z","timestamp":1782590628969,"version":"3.54.5"},"reference-count":69,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2025,6,24]],"date-time":"2025-06-24T00:00:00Z","timestamp":1750723200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"R\u00e9gion Centre Val de Loire"},{"DOI":"10.13039\/501100001665","name":"French National Research Agency","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001665","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,10,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Identifying disease-related molecular signatures that can be used as biomarkers is critical for the development of preclinical therapies for amyotrophic lateral sclerosis (ALS). In this study, we focused on the Prp-TDP-43A315T transgenic mouse model of ALS to explore peripheral and central molecular alterations associated with disease progression. Prp-TDP-43A315T transgenic (Tg) and C57BL\/6J wild-type mice were monitored from 50 to 400 postnatal days. One cohort assessed phenotypic parameters and MRI activity at 3 timepoints, ie, before (T0), at disease onset (T1), and at end-stage (T2). A second cohort validated findings from the first using omics analyses of tissues to examine ALS-related markers. Tg mice showed reduced body weight, decreased grip strength and tail position, and increased gait impairment at T1. Changes in (p)TDP-43, NRF2, GFAP, and pAMPK expression were noted in brain samples from the second cohort at T1. Metabolomic and lipidomic analyses revealed shifts in specific molecules in the brain and muscle of Tg mice. These data highlight individual differences in ALS pathology and adaptive responses to TDP-43-induced damage. This model provides valuable insights into TDP-43 proteinopathies and presents an innovative method for analyzing pathophysiological pathways through dried blood spot analysis, thereby expanding its applicability across various research fields.<\/jats:p>","DOI":"10.1093\/jnen\/nlaf071","type":"journal-article","created":{"date-parts":[[2025,6,2]],"date-time":"2025-06-02T07:23:58Z","timestamp":1748849038000},"page":"911-927","source":"Crossref","is-referenced-by-count":3,"title":["Identification of disease-associated molecular signatures in the Prp-TDP-43A315T mouse model of ALS: Toward preclinical biomarker development"],"prefix":"10.1093","volume":"84","author":[{"given":"Yara","family":"Al Ojaimi","sequence":"first","affiliation":[{"name":"Universit\u00e9 de Tours, INSERM, Imaging Brain & Neuropsychiatry iBraiN U1253, 37032, Tours, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samira","family":"Osman","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Tours, INSERM, Imaging Brain & Neuropsychiatry iBraiN U1253, 37032, Tours, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hugo","family":"Alarcan","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Tours, INSERM, Imaging Brain & Neuropsychiatry iBraiN U1253, 37032, Tours, France"},{"name":"CHU de Tours, Service de Biochimie et Biologie Mol\u00e9culaire, Tours, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Emond","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Tours, INSERM, Imaging Brain & Neuropsychiatry iBraiN U1253, 37032, Tours, France"},{"name":"CHU de Tours, Service de M\u00e9decine Nucl\u00e9aire In Vitro, Tours, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte","family":"Veyrat-Durebex","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Tours, INSERM, Imaging Brain & Neuropsychiatry iBraiN U1253, 37032, Tours, France"},{"name":"CHU de Tours, Service de Biochimie et Biologie Mol\u00e9culaire, Tours, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Debora","family":"Lanznaster","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Tours, INSERM, Imaging Brain & Neuropsychiatry iBraiN U1253, 37032, Tours, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Meme","sequence":"additional","affiliation":[{"name":"CBM, Orl\u00e9ans ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rudy","family":"Clemencon","sequence":"additional","affiliation":[{"name":"CBM, Orl\u00e9ans 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France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Vourc\u2019h","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Tours, INSERM, Imaging Brain & Neuropsychiatry iBraiN U1253, 37032, Tours, France"},{"name":"CHU de Tours, Service de Biochimie et Biologie Mol\u00e9culaire, Tours, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabienne","family":"Masse","sequence":"additional","affiliation":[{"name":"Key-Obs, Orl\u00e9ans, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabrice","family":"Trovero","sequence":"additional","affiliation":[{"name":"Key-Obs, Orl\u00e9ans, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6107-0035","authenticated-orcid":false,"given":"H\u00e9l\u00e8ne","family":"Blasco","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Tours, INSERM, Imaging Brain & Neuropsychiatry iBraiN U1253, 37032, Tours, France"},{"name":"CHU de Tours, Service de Biochimie et Biologie Mol\u00e9culaire, Tours, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2025,6,24]]},"reference":[{"key":"2025092317020656400_nlaf071-B1","doi-asserted-by":"crossref","first-page":"510","DOI":"10.1136\/jnnp-2020-325014","article-title":"Value of systematic genetic screening of patients with amyotrophic lateral sclerosis","volume":"92","author":"Shepheard","year":"2021","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"2025092317020656400_nlaf071-B2","doi-asserted-by":"crossref","first-page":"17071","DOI":"10.1038\/nrdp.2017.71","article-title":"Amyotrophic lateral sclerosis","volume":"3","author":"Hardiman","year":"2017","journal-title":"Nat Rev Dis Primers"},{"key":"2025092317020656400_nlaf071-B3","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1002\/ana.21147","article-title":"Pathological TDP-43 distinguishes sporadic amyotrophic 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