Department of Otolaryngology, Chang Gung Memorial Hospital at Chia-yi, Taiwan, ROC.
Hear Res. 2010 Jun 14;265(1-2):77-82. doi: 10.1016/j.heares.2010.02.008. Epub 2010 Feb 23.
Mutations of the GJB4 gene, encoding connexin 30.3 (CX30.3), are associated with skin disorders. Recently, this gene was also detected in deaf individuals without skin disorders. However, the functional roles of CX30.3 in the cochlea remain unclear. A primary step toward understanding the role of CX30.3 in hearing and its dysfunction is the documentation of its cellular and sub-cellular locations within the cochlea. In the present study, we located and determined the cellular expression of Cx30.3 within the rat cochlea by using a polyclonal anti-Cx30.3 antibody. Expression of the Cx30.3 protein was detected in the spiral limbus, spiral ligament, spiral ganglion, and stria vascularis by immunohistochemistry and reverse transcription-polymerase chain reaction (RT-PCR) analyses. Our results indicate the presence and localization of Cx30.3 in the rat cochlea. Knowledge of the spatial distribution of Cx30.3 will provide important insights into its role in the cochleae and normal auditory function.
GJB4 基因突变,编码连接蛋白 30.3(CX30.3),与皮肤疾病有关。最近,该基因也在没有皮肤疾病的耳聋个体中被检测到。然而,CX30.3 在耳蜗中的功能作用仍不清楚。了解 CX30.3 在听力及其功能障碍中的作用的一个首要步骤是记录其在耳蜗中的细胞和亚细胞位置。在本研究中,我们通过使用多克隆抗-Cx30.3 抗体定位并确定了大鼠耳蜗中的 Cx30.3 的细胞表达。免疫组织化学和逆转录-聚合酶链反应(RT-PCR)分析检测到 CX30.3 蛋白在螺旋边缘、螺旋韧带、螺旋神经节和血管纹中的表达。我们的结果表明 Cx30.3 存在于大鼠耳蜗中,并定位在那里。了解 Cx30.3 的空间分布将为其在耳蜗和正常听觉功能中的作用提供重要的见解。