{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T05:38:39Z","timestamp":1790746719909,"version":"4.1.0"},"reference-count":36,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2018,10,1]],"date-time":"2018-10-01T00:00:00Z","timestamp":1538352000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,10,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Fascin2 (FSCN2) is an actin cross-linking protein that is mainly localized in retinas and in the stereocilia of hair cells. Earlier studies showed that a deletion mutation in human FASCIN2 (FSCN2) gene could cause autosomal dominant retinitis pigmentosa. Recent studies have indicated that a missense mutation in mouse Fscn2 gene (R109H) can contribute to the early onset of hearing loss in DBA\/2J mice. To explore the function of the gene, Fscn2 was knocked out using TALEN (transcription activator-like effector nucleases) on the C57BL\/6J background. Four mouse strains with deletions of 1, 4, 5, and 41 nucleotides in the target region of Fscn2 were developed. F1 heterozygous (Fscn2+\/\u2212) mice carrying the same deletion of 41 nucleotides were mated to generate the Fscn2\u2212\/\u2212 mice. As a result, the Fscn2\u2212\/\u2212 mice showed progressive hearing loss, as measured in the elevation of auditory brainstem-response thresholds. The hearing impairment began at age 3 weeks at high-stimulus frequencies and became most severe at age 24 weeks. Moreover, degeneration of hair cells and loss of stereocilia were remarkable in Fscn2\u2212\/\u2212 mice, as revealed by F-actin staining and scanning electron microscopy. Furthermore, compared to the controls, the Fscn2\u2212\/\u2212 mice displayed significantly lower electroretinogram amplitudes and thinner retinas at 8, 16, and 24 weeks. These results demonstrate that, in C57BL\/6Jmice, Fscn2 is essential for maintaining ear and eye function and that a null mutation of Fscn2 leads to progressive hearing loss and retinal degeneration.<\/jats:p>","DOI":"10.1534\/g3.118.200405","type":"journal-article","created":{"date-parts":[[2018,10,3]],"date-time":"2018-10-03T14:06:05Z","timestamp":1538575565000},"page":"3221-3230","source":"Crossref","is-referenced-by-count":23,"title":["Null Mutation of the <i>Fascin2<\/i> Gene by TALEN Leading to Progressive Hearing Loss and Retinal Degeneration in C57BL\/6J Mice"],"prefix":"10.1093","volume":"8","author":[{"given":"Xiang","family":"Liu","sequence":"first","affiliation":[{"name":"Key Laboratory for Genetic Hearing Disorders in Shandong, Binzhou Medical, University, 346 Guanhai Road, Yantai 264003, Shandong, P. R. China"},{"name":"Department of Human Anatomy and Histology and Embryology, Binzhou Medical University, 346 Guanhai Road, Yantai 264003, Shandong, P. R. China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mengmeng","family":"Zhao","sequence":"additional","affiliation":[{"name":"Key Laboratory for Genetic Hearing Disorders in Shandong, Binzhou Medical, University, 346 Guanhai Road, Yantai 264003, Shandong, P. R. China"},{"name":"Department of Biochemistry and Molecular Biology, Binzhou Medical, University, 346 Guanhai Road, Yantai 264003, Shandong, P. R. China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi","family":"Xie","sequence":"additional","affiliation":[{"name":"Key Laboratory for Genetic Hearing Disorders in Shandong, Binzhou Medical, University, 346 Guanhai Road, Yantai 264003, Shandong, P. R. China"},{"name":"Department of Biochemistry and Molecular Biology, Binzhou Medical, University, 346 Guanhai Road, Yantai 264003, Shandong, P. R. 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