{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T08:48:43Z","timestamp":1771231723347,"version":"3.50.1"},"reference-count":103,"publisher":"The Company of Biologists","issue":"7","license":[{"start":{"date-parts":[[2025,4,11]],"date-time":"2025-04-11T00:00:00Z","timestamp":1744329600000},"content-version":"vor","delay-in-days":10,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100000268","name":"Biotechnology and Biological Sciences Research Council","doi-asserted-by":"crossref","award":["BB\/S005536\/1"],"award-info":[{"award-number":["BB\/S005536\/1"]}],"id":[{"id":"10.13039\/501100000268","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100000268","name":"Biotechnology and Biological Sciences Research Council","doi-asserted-by":"crossref","award":["BB\/V006339\/1"],"award-info":[{"award-number":["BB\/V006339\/1"]}],"id":[{"id":"10.13039\/501100000268","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100000268","name":"Biotechnology and Biological Sciences Research Council","doi-asserted-by":"crossref","award":["BB\/V006290\/1"],"award-info":[{"award-number":["BB\/V006290\/1"]}],"id":[{"id":"10.13039\/501100000268","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100000268","name":"Biotechnology and Biological Sciences Research Council","doi-asserted-by":"crossref","award":["BB\/X512047\/1"],"award-info":[{"award-number":["BB\/X512047\/1"]}],"id":[{"id":"10.13039\/501100000268","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100000272","name":"National Institute for Health and Care Research","doi-asserted-by":"crossref","award":["IS-BRC-1215-20006"],"award-info":[{"award-number":["IS-BRC-1215-20006"]}],"id":[{"id":"10.13039\/501100000272","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["R01 DE022065"],"award-info":[{"award-number":["R01 DE022065"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,4,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Branchio-otic (BOS) and branchio-oto-renal (BOR) syndromes are autosomal dominant disorders featuring multiple birth defects including ear, renal and branchial malformations. Mutations in the homeodomain transcription factor SIX1 and its co-factor EYA1 have been identified in about 50% of individuals with BOS or BOR, while causative mutations are unknown in the other half. We hypothesise that SIX1 target genes represent new BOS and BOR candidates. Using published transcriptomic and epigenomic data from chick ear progenitors, we first identify putative Six1 targets. Next, we provide evidence that Six1 directly regulates some of these candidates: Six1 binds to their enhancers, and functional experiments in Xenopus and chick confirm that Six1 controls their expression. Finally, we show that most putative chick Six1 targets are also expressed in the human developing ear and are associated with known deafness loci. Together, our results not only characterise the molecular mechanisms that mediate Six1 function in the developing ear, but also provide new candidates for human congenital deafness.<\/jats:p>","DOI":"10.1242\/dev.204533","type":"journal-article","created":{"date-parts":[[2025,4,11]],"date-time":"2025-04-11T08:42:01Z","timestamp":1744360921000},"source":"Crossref","is-referenced-by-count":5,"title":["Targets of the transcription factor Six1 identify previously unreported candidate deafness genes"],"prefix":"10.1242","volume":"152","author":[{"given":"Ramya","family":"Ranganathan","sequence":"first","affiliation":[{"name":"Centre for Craniofacial and Regenerative Biology, King's College London 1 , London SE1 9RT ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fereshteh","family":"Sari","sequence":"additional","affiliation":[{"name":"Centre for Craniofacial and Regenerative Biology, King's College London 1 , London SE1 9RT ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Scarlet Xiaoyan","family":"Wang","sequence":"additional","affiliation":[{"name":"Centre for Craniofacial and Regenerative Biology, King's College London 1 , London SE1 9RT ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexandre","family":"Thiery","sequence":"additional","affiliation":[{"name":"Centre for Craniofacial and Regenerative Biology, King's College London 1 , London SE1 9RT ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ailin Leticia","family":"Buzzi","sequence":"additional","affiliation":[{"name":"Centre for Craniofacial and Regenerative Biology, King's College London 1 , London SE1 9RT ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rosalinda","family":"Guerra","sequence":"additional","affiliation":[{"name":"Centre for Craniofacial and Regenerative Biology, King's College London 1 , London SE1 9RT ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sally A.","family":"Moody","sequence":"additional","affiliation":[{"name":"George Washington University School of Medicine and Health Sciences 2 Department of Anatomy & Cell Biology , , Washington, DC 20052 ,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7664-7917","authenticated-orcid":false,"given":"Andrea","family":"Streit","sequence":"additional","affiliation":[{"name":"Centre for Craniofacial and Regenerative Biology, King's College London 1 , London SE1 9RT ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"237","published-online":{"date-parts":[[2025,4,14]]},"reference":[{"key":"2025041404263700100_DEV204533C1","doi-asserted-by":"publisher","first-page":"re3","DOI":"10.1126\/scisignal.2005365","article-title":"The WNK-SPAK\/OSR1 pathway: master regulator of cation-chloride cotransporters","volume":"7","author":"Alessi","year":"2014","journal-title":"Sci. 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