{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T06:53:06Z","timestamp":1790837586077,"version":"4.1.0"},"reference-count":93,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2023,10,30]],"date-time":"2023-10-30T00:00:00Z","timestamp":1698624000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III CIBERNED","doi-asserted-by":"publisher","award":["PI21\/00372"],"award-info":[{"award-number":["PI21\/00372"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Fondo Europeo de Desarrollo Regional (FEDER)","award":["PI21\/00372"],"award-info":[{"award-number":["PI21\/00372"]}]},{"name":"Gobierno de Arag\u00f3n","award":["PI21\/00372"],"award-info":[{"award-number":["PI21\/00372"]}]},{"name":"Asahi Kasei Pharma (JRN)","award":["PI21\/00372"],"award-info":[{"award-number":["PI21\/00372"]}]},{"name":"Severo Ochoa Excellence Program","award":["PI21\/00372"],"award-info":[{"award-number":["PI21\/00372"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>The interaction of the activating transcription factor 6 (ATF6), a key effector of the unfolded protein response (UPR) in the endoplasmic reticulum, with the neuronal calcium sensor Downstream Regulatory Element Antagonist Modulator (DREAM) is a potential therapeutic target in neurodegeneration. Modulation of the ATF6\u2013DREAM interaction with repaglinide (RP) induced neuroprotection in a model of Huntington\u2019s disease. Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder with no cure, characterized by the progressive loss of motoneurons resulting in muscle denervation, atrophy, paralysis, and death. The aim of this work was to investigate the potential therapeutic significance of DREAM as a target for intervention in ALS. We found that the expression of the DREAM protein was reduced in the spinal cord of SOD1G93A mice compared to wild-type littermates. RP treatment improved motor strength and reduced the expression of the ALS progression marker collagen type XIX\u03b11 (Col19\u03b11 mRNA) in the quadriceps muscle in SOD1G93A mice. Moreover, treated SOD1G93A mice showed reduced motoneuron loss and glial activation and increased ATF6 processing in the spinal cord. These results indicate that the modulation of the DREAM\u2013ATF6 interaction ameliorates ALS symptoms in SOD1G93A mice.<\/jats:p>","DOI":"10.3390\/ijms242115783","type":"journal-article","created":{"date-parts":[[2023,10,30]],"date-time":"2023-10-30T13:20:07Z","timestamp":1698672007000},"page":"15783","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Repaglinide Induces ATF6 Processing and Neuroprotection in Transgenic SOD1G93A Mice"],"prefix":"10.3390","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6357-105X","authenticated-orcid":false,"given":"Rafael","family":"Gonzalo-Gobernado","sequence":"first","affiliation":[{"name":"National Centre for Biotechnology (CNB), Consejo Superior de Investigaciones Cient\u00edficas (CSIC), 28049 Madrid, Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red Enfermedades Neurodegenerativas (CIBERNED), Instituto de Salud Carlos III, 28029 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7277-4318","authenticated-orcid":false,"given":"Laura","family":"Moreno-Mart\u00ednez","sequence":"additional","affiliation":[{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red Enfermedades Neurodegenerativas (CIBERNED), Instituto de Salud Carlos III, 28029 Madrid, Spain"},{"name":"LAGENBIO, Faculty of Veterinary, University of Zaragoza, Miguel Servet 177, 50013 Zaragoza, Spain"},{"name":"Arag\u00f3n Health Research Institute (IIS Arag\u00f3n), Biomedical Research Centre of Arag\u00f3n (CIBA), 50009 Zaragoza, Spain"},{"name":"AgriFood Institute of Aragon-IA2 (UNIZAR-CITA), 50013 Zaragoza, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paz","family":"Gonz\u00e1lez","sequence":"additional","affiliation":[{"name":"National Centre for Biotechnology (CNB), Consejo Superior de Investigaciones Cient\u00edficas (CSIC), 28049 Madrid, Spain"},{"name":"Centro de 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50009 Zaragoza, Spain"},{"name":"AgriFood Institute of Aragon-IA2 (UNIZAR-CITA), 50013 Zaragoza, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabel","family":"Pidal-Ladr\u00f3n de Guevara","sequence":"additional","affiliation":[{"name":"National Centre for Biotechnology (CNB), Consejo Superior de Investigaciones Cient\u00edficas (CSIC), 28049 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Seisdedos","sequence":"additional","affiliation":[{"name":"National Centre for Biotechnology (CNB), Consejo Superior de Investigaciones Cient\u00edficas (CSIC), 28049 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-2786-0082","authenticated-orcid":false,"given":"Rodrigo","family":"D\u00edaz-Mu\u00f1oz","sequence":"additional","affiliation":[{"name":"National Centre for Biotechnology (CNB), Consejo Superior de Investigaciones Cient\u00edficas (CSIC), 28049 Madrid, 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