{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T01:50:02Z","timestamp":1790214602935,"version":"4.1.0"},"reference-count":30,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2020,6,1]],"date-time":"2020-06-01T00:00:00Z","timestamp":1590969600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,6,1]],"date-time":"2020-06-01T00:00:00Z","timestamp":1590969600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2019,11,27]],"date-time":"2019-11-27T00:00:00Z","timestamp":1574812800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003625","name":"Ministry of Health and Welfare","doi-asserted-by":"publisher","award":["HI16C0142"],"award-info":[{"award-number":["HI16C0142"]}],"id":[{"id":"10.13039\/501100003625","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["cell.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecular Therapy - Methods &amp; Clinical Development"],"published-print":{"date-parts":[[2020,6]]},"DOI":"10.1016\/j.omtm.2019.11.019","type":"journal-article","created":{"date-parts":[[2019,11,29]],"date-time":"2019-11-29T20:15:36Z","timestamp":1575058536000},"page":"188-197","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":11,"special_numbering":"C","title":["DNAJC14 Ameliorates Inner Ear Degeneration in the DFNB4 Mouse Model"],"prefix":"10.1016","volume":"17","author":[{"given":"Hye Ji","family":"Choi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hyun Jae","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jin Young","family":"Choi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ik Hyun","family":"Jeon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Byunghwa","family":"Noh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sushil","family":"Devkota","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Han-Woong","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seong Kug","family":"Eo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jae Young","family":"Choi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min Goo","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jinsei","family":"Jung","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.omtm.2019.11.019_bib1","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1038\/ng1297-411","article-title":"Pendred syndrome is caused by mutations in a putative sulphate transporter gene (PDS)","volume":"17","author":"Everett","year":"1997","journal-title":"Nat. Genet."},{"key":"10.1016\/j.omtm.2019.11.019_bib2","doi-asserted-by":"crossref","first-page":"F1345","DOI":"10.1152\/ajprenal.00487.2006","article-title":"Loss of cochlear HCO3- secretion causes deafness via endolymphatic acidification and inhibition of Ca2+ reabsorption in a Pendred syndrome mouse model","volume":"292","author":"Wangemann","year":"2007","journal-title":"Am. J. Physiol. Renal Physiol."},{"key":"10.1016\/j.omtm.2019.11.019_bib3","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1038\/7783","article-title":"The Pendred syndrome gene encodes a chloride-iodide transport protein","volume":"21","author":"Scott","year":"1999","journal-title":"Nat. Genet."},{"key":"10.1016\/j.omtm.2019.11.019_bib4","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1111\/j.1399-0004.2004.00386.x","article-title":"Genetic basis of hearing loss associated with enlarged vestibular aqueducts in Koreans","volume":"67","author":"Park","year":"2005","journal-title":"Clin. Genet."},{"key":"10.1016\/j.omtm.2019.11.019_bib5","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1136\/jmg.40.4.242","article-title":"Origins and frequencies of SLC26A4 (PDS) mutations in east and south Asians: global implications for the epidemiology of deafness","volume":"40","author":"Park","year":"2003","journal-title":"J.\u00a0Med. Genet."},{"key":"10.1016\/j.omtm.2019.11.019_bib6","doi-asserted-by":"crossref","first-page":"916","DOI":"10.1038\/sj.ejhg.5201073","article-title":"Distribution and frequencies of PDS (SLC26A4) mutations in Pendred syndrome and nonsyndromic hearing loss associated with enlarged vestibular aqueduct: a unique spectrum of mutations in Japanese","volume":"11","author":"Tsukamoto","year":"2003","journal-title":"Eur. J. Hum. Genet."},{"key":"10.1016\/j.omtm.2019.11.019_bib7","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1111\/j.1399-0004.2007.00862.x","article-title":"A distinct spectrum of SLC26A4 mutations in patients with enlarged vestibular aqueduct in China","volume":"72","author":"Wang","year":"2007","journal-title":"Clin. Genet."},{"key":"10.1016\/j.omtm.2019.11.019_bib8","doi-asserted-by":"crossref","first-page":"1060","DOI":"10.1097\/01.MLG.0000163339.61909.D0","article-title":"Prevalent SLC26A4 mutations in patients with enlarged vestibular aqueduct and\/or Mondini dysplasia: a unique spectrum of mutations in Taiwan, including a frequent founder mutation","volume":"115","author":"Wu","year":"2005","journal-title":"Laryngoscope"},{"key":"10.1016\/j.omtm.2019.11.019_bib9","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1186\/1479-5876-6-74","article-title":"Molecular etiology of hearing impairment in Inner Mongolia: mutations in SLC26A4 gene and relevant phenotype analysis","volume":"6","author":"Dai","year":"2008","journal-title":"J.\u00a0Transl. Med."},{"key":"10.1016\/j.omtm.2019.11.019_bib10","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1136\/jmg.2004.024208","article-title":"SLC26A4\/PDS genotype-phenotype correlation in hearing loss with enlargement of the vestibular aqueduct (EVA): evidence that Pendred syndrome and non-syndromic EVA are distinct clinical and genetic entities","volume":"42","author":"Pryor","year":"2005","journal-title":"J.\u00a0Med. Genet."},{"key":"10.1016\/j.omtm.2019.11.019_bib11","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/j.heares.2016.02.009","article-title":"Vestibular function is associated with residual low-frequency hearing loss in patients with bi-allelic mutations in the SLC26A4 gene","volume":"335","author":"Jung","year":"2016","journal-title":"Hear. Res."},{"key":"10.1016\/j.omtm.2019.11.019_bib12","doi-asserted-by":"crossref","first-page":"E286","DOI":"10.1002\/lary.25786","article-title":"Early deterioration of residual hearing in patients with SLC26A4 mutations","volume":"126","author":"Kim","year":"2016","journal-title":"Laryngoscope"},{"key":"10.1016\/j.omtm.2019.11.019_bib13","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1677\/JME-08-0175","article-title":"Functional characterization of wild-type and mutated pendrin (SLC26A4), the anion transporter involved in Pendred syndrome","volume":"43","author":"Dossena","year":"2009","journal-title":"J.\u00a0Mol. Endocrinol."},{"key":"10.1016\/j.omtm.2019.11.019_bib14","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1111\/cge.12273","article-title":"Correlation between genotype and phenotype in patients with bi-allelic SLC26A4 mutations","volume":"86","author":"Lee","year":"2014","journal-title":"Clin. Genet."},{"key":"10.1016\/j.omtm.2019.11.019_bib15","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1136\/jmg.2007.054635","article-title":"Heterogeneity in the processing defect of SLC26A4 mutants","volume":"45","author":"Yoon","year":"2008","journal-title":"J.\u00a0Med. Genet."},{"key":"10.1016\/j.omtm.2019.11.019_bib16","doi-asserted-by":"crossref","first-page":"11386","DOI":"10.1038\/ncomms11386","article-title":"The HSP70 co-chaperone DNAJC14 targets misfolded pendrin for unconventional protein secretion","volume":"7","author":"Jung","year":"2016","journal-title":"Nat. Commun."},{"key":"10.1016\/j.omtm.2019.11.019_bib17","doi-asserted-by":"crossref","first-page":"746","DOI":"10.1016\/j.cell.2011.07.021","article-title":"Rescue of \u0394F508-CFTR trafficking via a GRASP-dependent unconventional secretion pathway","volume":"146","author":"Gee","year":"2011","journal-title":"Cell"},{"key":"10.1016\/j.omtm.2019.11.019_bib18","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1016\/j.tcb.2016.11.007","article-title":"Pathways of Unconventional Protein Secretion","volume":"27","author":"Rabouille","year":"2017","journal-title":"Trends Cell Biol."},{"key":"10.1016\/j.omtm.2019.11.019_bib19","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1038\/35074561","article-title":"Regulation of transport of the dopamine D1 receptor by a new membrane-associated ER protein","volume":"3","author":"Bermak","year":"2001","journal-title":"Nat. Cell Biol."},{"key":"10.1016\/j.omtm.2019.11.019_bib20","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1007\/BF03402003","article-title":"The Chediak-Higashi protein interacts with SNARE complex and signal transduction proteins","volume":"8","author":"Tchernev","year":"2002","journal-title":"Mol. Med."},{"key":"10.1016\/j.omtm.2019.11.019_bib21","doi-asserted-by":"crossref","first-page":"e1001255","DOI":"10.1371\/journal.ppat.1001255","article-title":"Identification and characterization of the host protein DNAJC14 as a broadly active flavivirus replication modulator","volume":"7","author":"Yi","year":"2011","journal-title":"PLoS Pathog."},{"key":"10.1016\/j.omtm.2019.11.019_bib22","doi-asserted-by":"crossref","first-page":"11815","DOI":"10.1128\/JVI.01022-12","article-title":"Flavivirus replication complex assembly revealed by DNAJC14 functional mapping","volume":"86","author":"Yi","year":"2012","journal-title":"J.\u00a0Virol."},{"key":"10.1016\/j.omtm.2019.11.019_bib23","doi-asserted-by":"crossref","first-page":"3212","DOI":"10.1128\/JVI.03077-15","article-title":"Chaperone-Assisted Protein Folding Is Critical for Yellow Fever Virus NS3\/4A Cleavage and Replication","volume":"90","author":"Bozzacco","year":"2016","journal-title":"J.\u00a0Virol."},{"key":"10.1016\/j.omtm.2019.11.019_bib24","doi-asserted-by":"crossref","first-page":"e64906","DOI":"10.1371\/journal.pone.0064906","article-title":"Differences in the pathogenicity of the p.H723R mutation of the common deafness-associated SLC26A4 gene in humans and mice","volume":"8","author":"Lu","year":"2013","journal-title":"PLoS ONE"},{"key":"10.1016\/j.omtm.2019.11.019_bib25","doi-asserted-by":"crossref","first-page":"45973","DOI":"10.1038\/srep45973","article-title":"Genetic Predisposition to Sporadic Congenital Hearing Loss in a Pediatric Population","volume":"7","author":"Jung","year":"2017","journal-title":"Sci. Rep."},{"key":"10.1016\/j.omtm.2019.11.019_bib26","doi-asserted-by":"crossref","first-page":"e22150","DOI":"10.1371\/journal.pone.0022150","article-title":"Establishment of a knock-in mouse model with the SLC26A4 c.919-2A>G mutation and characterization of its pathology","volume":"6","author":"Lu","year":"2011","journal-title":"PLoS ONE"},{"key":"10.1016\/j.omtm.2019.11.019_bib27","doi-asserted-by":"crossref","first-page":"4516","DOI":"10.1172\/JCI59353","article-title":"Mouse model of enlarged vestibular aqueducts defines temporal requirement of Slc26a4 expression for hearing acquisition","volume":"121","author":"Choi","year":"2011","journal-title":"J.\u00a0Clin. Invest."},{"key":"10.1016\/j.omtm.2019.11.019_bib28","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1093\/hmg\/10.2.153","article-title":"Targeted disruption of mouse Pds provides insight about the inner-ear defects encountered in Pendred syndrome","volume":"10","author":"Everett","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.omtm.2019.11.019_bib29","doi-asserted-by":"crossref","first-page":"192","DOI":"10.4110\/in.2017.17.3.192","article-title":"Exacerbation of Japanese Encephalitis by CD11chi Dendritic Cell Ablation Is Associated with an Imbalance in Regulatory Foxp3+ and IL-17+CD4+ Th17 Cells and in Ly-6Chi and Ly-6Clo Monocytes","volume":"17","author":"Choi","year":"2017","journal-title":"Immune Netw."},{"key":"10.1016\/j.omtm.2019.11.019_bib30","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1016\/j.chom.2019.02.001","article-title":"Cleaved Cochlin Sequesters Pseudomonas aeruginosa and Activates Innate Immunity in the Inner Ear","volume":"25","author":"Jung","year":"2019","journal-title":"Cell Host Microbe"}],"container-title":["Molecular Therapy - Methods &amp; Clinical Development"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2329050119301445?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2329050119301445?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,11,6]],"date-time":"2025-11-06T09:29:49Z","timestamp":1762421389000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2329050119301445"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,6]]},"references-count":30,"alternative-id":["S2329050119301445"],"URL":"https:\/\/doi.org\/10.1016\/j.omtm.2019.11.019","relation":{},"ISSN":["2329-0501"],"issn-type":[{"value":"2329-0501","type":"print"}],"subject":[],"published":{"date-parts":[[2020,6]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"DNAJC14 Ameliorates Inner Ear Degeneration in the DFNB4 Mouse Model","name":"articletitle","label":"Article Title"},{"value":"Molecular Therapy - Methods & Clinical Development","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.omtm.2019.11.019","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2019 The Authors.","name":"copyright","label":"Copyright"}]}}