{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T20:16:59Z","timestamp":1790885819051,"version":"4.1.0"},"reference-count":95,"publisher":"American Physiological Society","issue":"4","funder":[{"DOI":"10.13039\/100000065","name":"HHS | NIH | National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["RF1NS114128"],"award-info":[{"award-number":["RF1NS114128"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"HHS | NIH | National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["R01NS114128"],"award-info":[{"award-number":["R01NS114128"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"HHS | NIH | National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["R01NS109150"],"award-info":[{"award-number":["R01NS109150"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Farber Family Foundation","award":["NA"],"award-info":[{"award-number":["NA"]}]},{"name":"Aldrich Foundation","award":["NA"],"award-info":[{"award-number":["NA"]}]}],"content-domain":{"domain":["journals.physiology.org"],"crossmark-restriction":true},"short-container-title":["American Journal of Physiology-Cell Physiology"],"published-print":{"date-parts":[[2025,10,1]]},"abstract":"<jats:p>A recent study using iPSC-derived cortical neurons reveals how neuronal activity drives gene dysregulation in C9ORF72-linked ALS\/FTD. We uncover synaptic dysfunction, peroxisomal dysregulation, and NPAS4-linked transcriptional shifts, highlighting key disease-modifying pathways. Could these insights pave the way for new therapeutic targets? Explore our research and generate your own discoveries using our interactive dataset included in the link in the article.<\/jats:p>","DOI":"10.1152\/ajpcell.00238.2025","type":"journal-article","created":{"date-parts":[[2025,8,28]],"date-time":"2025-08-28T16:54:31Z","timestamp":1756400071000},"page":"C1085-C1100","update-policy":"https:\/\/doi.org\/10.1152\/aps-crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Neuronal activity-dependent gene dysregulation in\n                    <i>C9orf72<\/i>\n                    i\n                    <sup>3<\/sup>\n                    Neuronal models of ALS\/FTD pathogenesis"],"prefix":"10.1152","volume":"329","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1573-7745","authenticated-orcid":false,"given":"Layla T.","family":"Ghaffari","sequence":"first","affiliation":[{"id":[{"id":"https:\/\/ror.org\/00ysqcn41","id-type":"ROR","asserted-by":"publisher"}],"name":"Thomas Jefferson University","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily A.","family":"Welebob","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/00ysqcn41","id-type":"ROR","asserted-by":"publisher"}],"name":"Thomas Jefferson University","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8309-5206","authenticated-orcid":false,"given":"Sarah E. B.","family":"Newton","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/00ysqcn41","id-type":"ROR","asserted-by":"publisher"}],"name":"Thomas Jefferson University","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2428-5656","authenticated-orcid":false,"given":"Ashley V.","family":"Boehringer","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/00ysqcn41","id-type":"ROR","asserted-by":"publisher"}],"name":"Thomas Jefferson University","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kelly L.","family":"Cyliax","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/00ysqcn41","id-type":"ROR","asserted-by":"publisher"}],"name":"Thomas Jefferson University","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piera","family":"Pasinelli","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/00ysqcn41","id-type":"ROR","asserted-by":"publisher"}],"name":"Thomas Jefferson University","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davide","family":"Trotti","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/00ysqcn41","id-type":"ROR","asserted-by":"publisher"}],"name":"Thomas Jefferson University","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5566-3707","authenticated-orcid":false,"given":"Aaron R.","family":"Haeusler","sequence":"additional","affiliation":[{"id":[{"id":"https:\/\/ror.org\/00ysqcn41","id-type":"ROR","asserted-by":"publisher"}],"name":"Thomas Jefferson University","place":["United States"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"24","reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2013.07.033"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1038\/nm.4490"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2013.10.015"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1038\/nature13124"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.17820"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.08881"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-013-1181-y"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2011.09.010"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2011.09.011"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.3389\/fnmol.2017.00035"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.3389\/fnmol.2022.1005112"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1038\/nn.4494"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1007\/s12035-014-8772-z"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1038\/nn.2900"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1038\/nature09033"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2018.10.013"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.neuro.31.060407.125631"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1038\/nature11860"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25047"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2015.2197"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.3988\/jcn.2013.9.2.65"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1016\/j.expneurol.2009.03.039"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awn071"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(10)61156-7"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1172\/JCI130988"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.15252\/emmm.202013131"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.15252\/emmm.201809423"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1016\/j.isci.2023.106959"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1002\/cpcb.51"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.1186\/s12868-017-0391-y"},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/bts635"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkz114"},{"key":"B33","doi-asserted-by":"publisher","DOI":"10.1186\/s13059-014-0550-8"},{"key":"B34","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btl056"},{"key":"B35","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btu333"},{"key":"B36","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awab171"},{"key":"B37","doi-asserted-by":"publisher","DOI":"10.4137\/JCNSD.S18103"},{"key":"B38","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.3007529"},{"key":"B39","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ejhg.5201783"},{"key":"B40","doi-asserted-by":"publisher","DOI":"10.1016\/j.tins.2010.02.004"},{"key":"B41","doi-asserted-by":"publisher","DOI":"10.1186\/s40478-022-01455-z"},{"key":"B42","doi-asserted-by":"publisher","DOI":"10.1038\/s41586-023-05711-7"},{"key":"B43","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.2738-17.2018"},{"key":"B44","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.1328-13.2014"},{"key":"B45","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.33092"},{"key":"B46","doi-asserted-by":"publisher","DOI":"10.1177\/1759091420974807"},{"key":"B47","doi-asserted-by":"publisher","DOI":"10.1007\/s12035-016-9773-x"},{"key":"B48","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.2672-10.2011"},{"key":"B49","doi-asserted-by":"publisher","DOI":"10.1038\/ng1001-166"},{"key":"B50","doi-asserted-by":"publisher","DOI":"10.1097\/NEN.0b013e318275df4b"},{"key":"B51","doi-asserted-by":"publisher","DOI":"10.3390\/biom10050760"},{"key":"B52","doi-asserted-by":"publisher","DOI":"10.1002\/mus.22217"},{"key":"B53","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1326-6-51"},{"key":"B54","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2021.109224"},{"key":"B55","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2013.08.003"},{"key":"B56","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2008.05486.x"},{"key":"B57","doi-asserted-by":"publisher","DOI":"10.3389\/fcell.2020.592164"},{"key":"B58","doi-asserted-by":"publisher","DOI":"10.1111\/jnc.15594"},{"key":"B59","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-319-55769-4_6"},{"key":"B60","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms6999"},{"key":"B61","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-022-09751-3"},{"key":"B62","doi-asserted-by":"publisher","DOI":"10.3389\/fnmol.2020.00117"},{"key":"B63","doi-asserted-by":"publisher","DOI":"10.1186\/s12920-022-01313-w"},{"key":"B64","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddaa106"},{"key":"B65","doi-asserted-by":"publisher","DOI":"10.3389\/fnmol.2022.89423"},{"key":"B66","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.00725.2024"},{"key":"B67","doi-asserted-by":"publisher","DOI":"10.3389\/fnins.2020.603373"},{"key":"B68","doi-asserted-by":"publisher","DOI":"10.1523\/ENEURO.0322-19.2020"},{"key":"B69","doi-asserted-by":"publisher","DOI":"10.1038\/s41593-020-00786-1"},{"key":"B70","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1722546115"},{"key":"B71","doi-asserted-by":"publisher","DOI":"10.1001\/jamapsychiatry.2016.2575"},{"key":"B72","doi-asserted-by":"publisher","DOI":"10.3390\/ph15040433"},{"key":"B73","doi-asserted-by":"publisher","DOI":"10.1016\/j.neulet.2021.136039"},{"key":"B74","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.51039"},{"key":"B75","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2012-304019"},{"key":"B76","doi-asserted-by":"publisher","DOI":"10.1186\/s13024-020-0359-8"},{"key":"B77","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-017-1760-4"},{"key":"B78","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-017-1713-y"},{"key":"B79","doi-asserted-by":"publisher","DOI":"10.3390\/ijms22042034"},{"key":"B80","doi-asserted-by":"publisher","DOI":"10.3389\/fcell.2020.00639"},{"key":"B81","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddp222"},{"key":"B82","doi-asserted-by":"publisher","DOI":"10.1038\/sj.cdd.4401384"},{"key":"B83","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.63.8.3"},{"key":"B84","doi-asserted-by":"publisher","DOI":"10.1016\/j.exer.2015.11.006"},{"key":"B85","doi-asserted-by":"publisher","DOI":"10.1016\/j.exer.2008.10.011"},{"key":"B86","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2990.2001.00310.x"},{"key":"B87","doi-asserted-by":"publisher","DOI":"10.3390\/ijms21176333"},{"key":"B88","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2021.05.020"},{"key":"B89","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.48363"},{"key":"B90","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.E16-01-0003"},{"key":"B91","doi-asserted-by":"publisher","DOI":"10.1186\/s40478-016-0324-5"},{"key":"B92","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-022-02470-z"},{"key":"B93","doi-asserted-by":"publisher","DOI":"10.1186\/s40478-018-0579-0"},{"key":"B94","doi-asserted-by":"publisher","DOI":"10.1186\/s40478-019-0812-5"},{"key":"B95","doi-asserted-by":"publisher","DOI":"10.15252\/emmm.201910722"}],"container-title":["American Journal of Physiology-Cell Physiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.physiology.org\/doi\/pdf\/10.1152\/ajpcell.00238.2025","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T13:41:52Z","timestamp":1761054112000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.physiology.org\/doi\/10.1152\/ajpcell.00238.2025"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,1]]},"references-count":95,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2025,10,1]]}},"alternative-id":["10.1152\/ajpcell.00238.2025"],"URL":"https:\/\/doi.org\/10.1152\/ajpcell.00238.2025","relation":{"has-preprint":[{"id-type":"doi","id":"10.1101\/2025.01.27.632228","asserted-by":"object"}]},"ISSN":["0363-6143","1522-1563"],"issn-type":[{"value":"0363-6143","type":"print"},{"value":"1522-1563","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10,1]]},"assertion":[{"value":"2025-03-18","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-04-11","order":1,"name":"revised","label":"Revised","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-08-15","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-09-26","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}