{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T17:53:18Z","timestamp":1788544398949,"version":"build-2803163510"},"reference-count":35,"publisher":"The Company of Biologists","issue":"2","license":[{"start":{"date-parts":[[2020,9,5]],"date-time":"2020-09-05T00:00:00Z","timestamp":1599264000000},"content-version":"vor","delay-in-days":947,"URL":"http:\/\/creativecommons.org\/licenses\/by\/3.0"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"crossref","award":["81500793"],"award-info":[{"award-number":["81500793"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"crossref","award":["81570923"],"award-info":[{"award-number":["81570923"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"crossref","award":["81470696"],"award-info":[{"award-number":["81470696"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"crossref","award":["81500795"],"award-info":[{"award-number":["81500795"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,2,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Mutations in the GJB2 gene [which encodes connexin 26 (Cx26)] are the most common causes of hereditary hearing loss in humans, and previous studies showed postnatal development arrest of the organ of Corti in different Cx26-null mouse models. To explore the pathological changes and the mechanism behind the cochlear abnormalities in these mice further, we established transgenic mouse models by conditional knockdown of cochlear Cx26 at postnatal day (P) 0 and P8. Auditory brainstem responses were recorded and the morphological features in the organ of Corti were analyzed 18\u2005days after Cx26 knockdown. Mice in the P0 knockdown group displayed severe hearing loss at all frequencies, whereas mice in the P8 knockdown group showed nearly normal hearing. In the P8 knockdown group, the organ of Corti displayed normal architecture, and no ultrastructural changes were observed. In the P0 knockdown group, the phalangeal processes of Deiter's cells did not develop into finger-like structures, and the formation of microtubules in the pillar cells was significantly reduced; moreover, the amount of acetylated \u03b1-tubulin was reduced in pillar cells. Our results indicate that Gjb2 participates in postnatal development of the cytoskeleton in pillar cells during structural maturation of the organ of Corti. In P0 knockdown mice, the reduction in microtubules in pillar cells might be responsible for the failure of the tunnel of Corti to open, and the malformed phalangeal processes might negatively affect the supporting framework of the organ of Corti, which would be a new mechanism of Gjb2-related hearing loss.<\/jats:p>","DOI":"10.1242\/dmm.033019","type":"journal-article","created":{"date-parts":[[2018,1,20]],"date-time":"2018-01-20T01:10:53Z","timestamp":1516410653000},"source":"Crossref","is-referenced-by-count":31,"title":["Developmental abnormalities in supporting cell phalangeal processes and cytoskeleton in the <i>Gjb2<\/i> knockdown mouse model"],"prefix":"10.1242","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9579-8080","authenticated-orcid":false,"given":"Sen","family":"Chen","sequence":"first","affiliation":[{"name":"Union Hospital, Tongji Medical College, Huazhong University of Science and Technology 1 Department of Otorhinolaryngology , , Wuhan 430022 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Le","family":"Xie","sequence":"additional","affiliation":[{"name":"Union Hospital, Tongji Medical College, Huazhong University of Science and Technology 1 Department of Otorhinolaryngology , , Wuhan 430022 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kai","family":"Xu","sequence":"additional","affiliation":[{"name":"Union Hospital, Tongji Medical College, Huazhong University of Science and Technology 1 Department of Otorhinolaryngology , , Wuhan 430022 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hai-Yan","family":"Cao","sequence":"additional","affiliation":[{"name":"Union Hospital, Tongji Medical College, Huazhong University of Science and Technology 2 Department of Ultrasound , , Wuhan 430022 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xia","family":"Wu","sequence":"additional","affiliation":[{"name":"Union Hospital, Tongji Medical College, Huazhong University of Science and Technology 1 Department of Otorhinolaryngology , , Wuhan 430022 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiao-Xiang","family":"Xu","sequence":"additional","affiliation":[{"name":"Union Hospital, Tongji Medical College, Huazhong University of Science and Technology 1 Department of Otorhinolaryngology , , Wuhan 430022 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1771-3715","authenticated-orcid":false,"given":"Yu","family":"Sun","sequence":"additional","affiliation":[{"name":"Union Hospital, Tongji Medical College, Huazhong University of Science and Technology 1 Department of Otorhinolaryngology , , Wuhan 430022 , China"},{"name":"Institute of Otorhinolaryngology, Tongji Medical College, Huazhong University of Science and Technology 3 , Wuhan 430022 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5410-2297","authenticated-orcid":false,"given":"Wei-Jia","family":"Kong","sequence":"additional","affiliation":[{"name":"Union Hospital, Tongji Medical College, Huazhong University of Science and Technology 1 Department of Otorhinolaryngology , , Wuhan 430022 , China"},{"name":"Institute of Otorhinolaryngology, Tongji Medical College, Huazhong University of Science and Technology 3 , Wuhan 430022 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2018,2,26]]},"reference":[{"key":"2024061302115803900_DMM033019C1","doi-asserted-by":"publisher","first-page":"362","DOI":"10.1016\/S0006-291X(03)01166-5","article-title":"Connexins 26 and 30 are co-assembled to form gap junctions in the cochlea of mice","volume":"307","author":"Ahmad","year":"2003","journal-title":"Biochem. 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