{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T07:11:22Z","timestamp":1791443482702,"version":"4.3.3"},"reference-count":25,"publisher":"American Society for Clinical Investigation","issue":"3","license":[{"start":{"date-parts":[[2025,2,3]],"date-time":"2025-02-03T00:00:00Z","timestamp":1738540800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,2,3]]},"abstract":"<jats:p>\n                    Sensorineural hearing loss (SNHL) is the most prevalent form of permanent hearing impairment, arising from factors such as aging, exposure to loud noise, disease, ototoxic medications, and genetic mutations. Despite extensive research, effective treatments or cures for SNHL remain elusive. In this issue of the\n                    <jats:italic>JCI<\/jats:italic>\n                    , Lee et al. reveal a link between mutations in\n                    <jats:italic>ATF6<\/jats:italic>\n                    and SNHL in patients with achromatopsia. The study also shows that\n                    <jats:italic>Atf6<\/jats:italic>\n                    -deficient (\n                    <jats:italic>\n                      Atf6\n                      <jats:sup>\u2013\/\u2013<\/jats:sup>\n                    <\/jats:italic>\n                    ) mice exhibit disorganized stereocilia and age-related loss of outer hair cells. Additionally, the researchers show that\n                    <jats:italic>Atf6<\/jats:italic>\n                    is critical for cochlear hair cell function. Mice lacking\n                    <jats:italic>Atf6<\/jats:italic>\n                    expression experienced ER stress, which ultimately led to SNHL. Collectively, these findings enhance our understanding of the emerging role of protein homeostasis and ER stress in the pathogenesis of SNHL.\n                  <\/jats:p>","DOI":"10.1172\/jci188708","type":"journal-article","created":{"date-parts":[[2025,2,2]],"date-time":"2025-02-02T14:00:45Z","timestamp":1738504845000},"source":"Crossref","is-referenced-by-count":4,"title":["A deafness-blindness syndrome results from ATF6-based disruption of the unfolded protein response"],"prefix":"10.1172","volume":"135","author":[{"given":"Yuvraj","family":"Joshi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey N.","family":"Savas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"232","published-online":{"date-parts":[[2025,2,3]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.12-12-04575.1992"},{"issue":"3","key":"B2","doi-asserted-by":"crossref","DOI":"10.1172\/JCI175562","article-title":"Mutations in unfolded protein response regulator ATF6 cause hearing and vision loss syndrome","volume":"135","author":"Lee","year":"2025","journal-title":"J Clin Invest"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1016\/j.heliyon.2024.e25937"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1038\/ng.3319"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1606387114"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1038\/nature06091"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddi490"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1038\/374060a0"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1038\/83660"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1086\/323610"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1038\/ng.2426"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1111\/cge.13170"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1002\/ajim.20223"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a033951"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1126\/science.1141448"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1016\/j.heares.2023.108821"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.15252\/embr.201847097"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1016\/j.exger.2015.07.003"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1172\/JCI97498"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2021.109758"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2020.108431"},{"key":"B22","doi-asserted-by":"crossref","unstructured":"Park Y, et al. Noise induces intercellular Ca2+ signaling waves and the unfolded protein response in the hearing cochlea. bioRxiv. https:\/\/doi.org\/10.1101\/2024.06.05.597671","DOI":"10.1101\/2024.06.05.597671"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-03-143107"},{"issue":"3","key":"B24","first-page":"180","article-title":"Single-cell transcriptomic atlas of mouse cochlear aging","volume":"14","author":"Sun","year":"2023","journal-title":"Protein Cell"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1038\/nn.2330"}],"container-title":["Journal of Clinical Investigation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.jci.org\/articles\/view\/188708\/files\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T22:49:35Z","timestamp":1779230975000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.jci.org\/articles\/view\/188708"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,2,3]]},"references-count":25,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2024,12,2]]},"published-print":{"date-parts":[[2025,2,3]]}},"alternative-id":["10.1172\/JCI188708"],"URL":"https:\/\/doi.org\/10.1172\/jci188708","relation":{},"ISSN":["1558-8238"],"issn-type":[{"value":"1558-8238","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,2,3]]},"article-number":"e188708"}}