{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T04:46:06Z","timestamp":1791521166638,"version":"4.3.4"},"reference-count":77,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2021,6,11]],"date-time":"2021-06-11T00:00:00Z","timestamp":1623369600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100009017","name":"ALS Canada","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100009017","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Brain Canada Hudson Translational Team"},{"DOI":"10.13039\/100005202","name":"Muscular Dystrophy Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005202","id-type":"DOI","asserted-by":"publisher"}]},{"name":"James Hunter ALS Initiative"},{"name":"ALS Association Milton Safenowitz Postdoctoral Fellowship"},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01-NS097542"],"award-info":[{"award-number":["R01-NS097542"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"National Institute for Aging","award":["P30 AG053760"],"award-info":[{"award-number":["P30 AG053760"]}]},{"name":"FRQS Doctoral Studentship"},{"name":"FRQS Research Scholars"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,12,16]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>TDP-43 nuclear depletion and concurrent cytoplasmic accumulation in vulnerable neurons is a hallmark feature of progressive neurodegenerative proteinopathies such as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Cellular stress signalling and stress granule dynamics are now recognized to play a role in ALS\/FTD pathogenesis. Defective stress granule assembly is associated with increased cellular vulnerability and death. Ras-GAP SH3-domain-binding protein 1 (G3BP1) is a critical stress granule assembly factor.<\/jats:p>\n               <jats:p>Here, we define that TDP-43 stabilizes G3BP1 transcripts via direct binding of a highly conserved cis regulatory element within the 3\u02b9 untranslated region. Moreover, we show in vitro and in vivo that nuclear TDP-43 depletion is sufficient to reduce G3BP1 protein levels. Finally, we establish that G3BP1 transcripts are reduced in ALS\/FTD patient neurons bearing TDP-43 cytoplasmic inclusions\/nuclear depletion.<\/jats:p>\n               <jats:p>Thus, our data indicate that, in ALS\/FTD, there is a compromised stress granule response in disease-affected neurons due to impaired G3BP1 mRNA stability caused by TDP-43 nuclear depletion. These data implicate TDP-43 and G3BP1 loss of function as contributors to disease.<\/jats:p>","DOI":"10.1093\/brain\/awab217","type":"journal-article","created":{"date-parts":[[2021,6,9]],"date-time":"2021-06-09T03:39:41Z","timestamp":1623209981000},"page":"3461-3476","source":"Crossref","is-referenced-by-count":74,"title":["TDP-43 stabilizes <i>G3BP1<\/i> mRNA: relevance to amyotrophic lateral sclerosis\/frontotemporal dementia"],"prefix":"10.1093","volume":"144","author":[{"given":"Hadjara","family":"Sidib\u00e9","sequence":"first","affiliation":[{"name":"Department of Neurosciences, Universit\u00e9 de Montr\u00e9al, Montr\u00e9al, QC H3A 0E8, Canada"},{"name":"CHUM Research Center, Montr\u00e9al, QC H2X 0A9, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yousra","family":"Khalfallah","sequence":"additional","affiliation":[{"name":"CHUM Research Center, Montr\u00e9al, QC H2X 0A9, Canada"},{"name":"Department of Biochemistry, Universit\u00e9 de Montr\u00e9al, 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