Dernedde Jens, Weise Christoph, Müller Eva-Christina, Hagiwara Akira, Bachmann Sebastian, Suzuki Mamoru, Reutter Werner, Tauber Rudolf, Scherer Hans
Institut für Laboratoriumsmedizin, Klinische Chemie und Pathobiochemie, Charité -Universitätsmedizin Berlin, Berlin, Germany.
Institut für Chemie und Biochemie, Freie Universität Berlin, Berlin, Germany.
PLoS One. 2014 Nov 4;9(11):e111917. doi: 10.1371/journal.pone.0111917. eCollection 2014.
The extracellular membranes of the inner ear are essential constituents to maintain sensory functions, the cupula for sensing torsional movements of the head, the otoconial membrane for sensing linear movements and accelerations like gravity, and the tectorial membrane in the cochlea for hearing. So far a number of structural proteins have been described, but for the gelatinous cupula precise data are missing. Here, we describe for the first time a major proteinogenic component of the cupula structure with an apparent molecular mass of 45 kDa from salmon. Analyses of respective peptides revealed highly conserved amino-acid sequences with identity to zona pellucida-like domain proteins. Immunohistochemistry studies localized the protein in the ampulla of the inner ear from salmon and according to its anatomical appearance we identified this glycoprotein as Cupulin. Future research on structure and function of zona pellucida-like domain proteins will enhance our knowledge of inner ear diseases, like sudden loss of vestibular function and other disturbances.
内耳的细胞外膜是维持感觉功能的重要组成部分,其中壶腹嵴用于感知头部的扭转运动,耳石膜用于感知线性运动和诸如重力等加速度,而耳蜗中的覆膜则用于听觉。到目前为止,已经描述了许多结构蛋白,但关于凝胶状壶腹嵴的精确数据仍然缺失。在这里,我们首次描述了来自鲑鱼的壶腹嵴结构中一种主要的蛋白质成分,其表观分子量为45 kDa。对相应肽段的分析揭示了与透明带样结构域蛋白具有同源性的高度保守的氨基酸序列。免疫组织化学研究将该蛋白定位在鲑鱼内耳的壶腹中,根据其解剖学外观,我们将这种糖蛋白鉴定为杯蛋白。未来对透明带样结构域蛋白的结构和功能的研究将增进我们对诸如前庭功能突然丧失和其他紊乱等内耳疾病的了解。