软骨表皮生长因子在耳蜗支持细胞中特异性表达，是正常听力所必需的。
Epiphycan is specifically expressed in cochlear supporting cells and is necessary for normal hearing.
作者信息
Hanada Yukiko, Nakamura Yukiko, Ishida Yusuke, Takimoto Yasumitsu, Taniguchi Manabu, Ozono Yoshiyuki, Koyama Yoshihisa, Morihana Tetsuo, Imai Takao, Ota Yumi, Sato Takashi, Inohara Hidenori, Shimada Shoichi
机构信息
Department of Neuroscience and Cell Biology, Graduate School of Medicine, Osaka University, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan; Department of Otorhinolaryngology-Head and Neck Surgery, Graduate School of Medicine, Osaka University, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan.
Department of Neuroscience and Cell Biology, Graduate School of Medicine, Osaka University, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan.
出版信息
Biochem Biophys Res Commun. 2017 Oct 21;492(3):379-385. doi: 10.1016/j.bbrc.2017.08.092. Epub 2017 Aug 31.
The study of inner ear specific transcripts has revealed novel information about hereditary hearing loss and a mechanism of normal hearing. In this study, by analyzing a published cDNA library, we focused on Epiphycan (Epyc), a member of the small leucine-rich repeat proteoglycan family, whose transcript is enriched in the inner ear. Epyc mRNA was expressed abundantly and specifically in adult mice cochleae and was localized in supporting cells within the organ of Corti of both neonatal and adult mice. To examine the function of Epyc, we generated Epyc knockout (KO) mice using the CRISPR/Cas9 system. Epyc KO mice cochleae exhibited normal morphology. However, measurement of the auditory brain-stem response in Epyc KO mice revealed an elevated hearing threshold above 16 kHz frequency. This study suggests that Epyc is necessary for normal auditory function.
对内耳特异性转录本的研究揭示了有关遗传性听力损失的新信息以及正常听力的机制。在本研究中，通过分析已发表的cDNA文库，我们聚焦于富含亮氨酸的小分子重复蛋白聚糖家族成员Epiphycan（Epyc），其转录本在内耳中富集。Epyc mRNA在成年小鼠耳蜗中大量且特异性表达，并定位于新生和成年小鼠柯蒂氏器内的支持细胞中。为了研究Epyc的功能，我们使用CRISPR/Cas9系统构建了Epyc基因敲除（KO）小鼠。Epyc基因敲除小鼠的耳蜗形态正常。然而，对Epyc基因敲除小鼠听觉脑干反应的测量显示，在频率高于16kHz时听力阈值升高。这项研究表明，Epyc对正常听觉功能是必需的。
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