{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T12:52:49Z","timestamp":1792155169074,"version":"4.4.0"},"reference-count":49,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2019,7,4]],"date-time":"2019-07-04T00:00:00Z","timestamp":1562198400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100007533","name":"Kobayashi Foundation for Cancer Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007533","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Genes to Cells"],"published-print":{"date-parts":[[2019,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Zinc\u2010finger transcription factors GATA2 and GATA3 are both expressed in the developing inner ear, although their overlapping versus distinct activities in adult definitive inner ear are not well understood. We show here that GATA2 and GATA3 are co\u2010expressed in cochlear spiral ganglion cells and redundantly function in the maintenance of spiral ganglion cells and auditory neural circuitry. Notably, <jats:italic>Gata2<\/jats:italic> and <jats:italic>Gata3<\/jats:italic> compound heterozygous mutant mice had a diminished number of spiral ganglion cells due to enhanced apoptosis, which resulted in progressive hearing loss. The decrease in spiral ganglion cellularity was associated with lowered expression of neurotrophin receptor TrkC that is an essential factor for spiral ganglion cell survival. We further show that <jats:italic>Gata2<\/jats:italic> null mutants that additionally bear a <jats:italic>Gata2<\/jats:italic> YAC (yeast artificial chromosome) that counteracts the lethal hematopoietic deficiency due to complete <jats:italic>Gata2<\/jats:italic> loss nonetheless failed to complement the deficiency in neonatal spiral ganglion neurons. Furthermore, cochlea\u2010specific <jats:italic>Gata2<\/jats:italic> deletion mice also had fewer spiral ganglion cells and resultant hearing impairment. These results show that GATA2 and GATA3 redundantly function to maintain spiral ganglion cells and hearing. We propose possible mechanisms underlying hearing loss in human <jats:italic>GATA2\u2010<\/jats:italic> or <jats:italic>GATA3<\/jats:italic>\u2010related genetic disorders.<\/jats:p>","DOI":"10.1111\/gtc.12705","type":"journal-article","created":{"date-parts":[[2019,5,29]],"date-time":"2019-05-29T13:51:06Z","timestamp":1559137866000},"page":"534-545","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Spiral ganglion cell degeneration\u2010induced deafness as a consequence of reduced GATA factor activity"],"prefix":"10.1111","volume":"24","author":[{"given":"Tomofumi","family":"Hoshino","sequence":"first","affiliation":[{"name":"Department of Otolaryngology, Faculty of Medicine University of Tsukuba Tsukuba Japan"},{"name":"Department of Medical Biochemistry Tohoku University Graduate School of Medicine Sendai 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