Otolith tethering in the zebrafish otic vesicle requires Otogelin and α-Tectorin.
Otolith tethering in the zebrafish otic vesicle requires Otogelin and α-Tectorin.
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DOI：
10.1242/dev.116632
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发表时间：
2015-03-15
期刊：
影响因子：
--
通讯作者：
Whitfield TT
中科院分区：
文献类型：
--
作者：
Stooke-Vaughan GA;Obholzer ND;Baxendale S;Megason SG;Whitfield TT
耳石是生物矿化结构，对鱼类的平衡和听力很重要。它们在哺乳动物内耳中的对应物，耳蜗，主要具有前庭功能。耳石和耳郭形成于感觉斑疹之上，并附着在耳石膜上，耳石膜是一种胶质细胞外基质，在耳石和下面的感觉上皮之间提供物理耦合。在这项研究中，我们已经确定了斑马鱼耳朵中耳石系结所需的两种蛋白质，并提出该过程至少有两个阶段：播种和维持。在最初的植入步骤中，耳石前体颗粒直接系在毛细胞纤毛的尖端，在爱因斯坦（eis）突变体中没有发生。eis中被破坏的基因是otogelin (otog)；人类OTOG基因突变最近被确定为耳聋和前庭功能障碍的病因（DFNB18B）。在幼虫后期，耳石粘附在囊状黄斑的维持依赖于耳石蛋白α (tecta)功能，该功能在滚石突变体中被破坏。α-Tectorin （Tecta）是哺乳动物耳蜗被覆膜的主要成分。人类TECTA基因突变可导致显性（DFNA8/12）或隐性（DFNB21）形式的耳聋。我们的研究结果表明，细胞外耳膜的组成在哺乳动物和鱼类之间高度保守，加强了斑马鱼是研究耳聋和前庭疾病的优秀模型系统的观点。摘要：斑马鱼耳石的系结对鱼类的平衡和听力很重要，它分两个阶段发生，这两个阶段依赖于与人类耳聋有关的基因。
Otoliths are biomineralised structures important for balance and hearing in fish. Their counterparts in the mammalian inner ear, otoconia, have a primarily vestibular function. Otoliths and otoconia form over sensory maculae and are attached to the otolithic membrane, a gelatinous extracellular matrix that provides a physical coupling between the otolith and the underlying sensory epithelium. In this study, we have identified two proteins required for otolith tethering in the zebrafish ear, and propose that there are at least two stages to this process: seeding and maintenance. The initial seeding step, in which otolith precursor particles tether directly to the tips of hair cell kinocilia, fails to occur in the einstein (eis) mutant. The gene disrupted in eis is otogelin (otog); mutations in the human OTOG gene have recently been identified as causative for deafness and vestibular dysfunction (DFNB18B). At later larval stages, maintenance of otolith tethering to the saccular macula is dependent on tectorin alpha (tecta) function, which is disrupted in the rolling stones (rst) mutant. α-Tectorin (Tecta) is a major constituent of the tectorial membrane in the mammalian cochlea. Mutations in the human TECTA gene can cause either dominant (DFNA8/12) or recessive (DFNB21) forms of deafness. Our findings indicate that the composition of extracellular otic membranes is highly conserved between mammals and fish, reinforcing the view that the zebrafish is an excellent model system for the study of deafness and vestibular disease. Summary: The tethering of zebrafish otoliths, which are important for balance and hearing in fish, occurs in two stages that are dependent on genes that have been implicated in human deafness.
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影响因子：
2.5
作者：
Clendenon, Sherry G.;Shah, Bijal;Miller, Caroline A.;Schmeisser, Glen;Walter, Amanda;Gattone, Vincent H., II;Barald, Kate F.;Liu, Qin;Marrs, James A.
通讯作者：
Marrs, James A.
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4.4
作者：
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影响因子：
1.2
作者：
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通讯作者：
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影响因子：
30.8
作者：
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通讯作者：
Müller, U
影响因子：
2.9
作者：
Lundberg, Yunxia Wang;Zhao, Xing;Yamoah, Ebenezer N.
通讯作者：
Yamoah, Ebenezer N.