Lundberg Yunxia Wang, Xu Yinfang, Thiessen Kevin D, Kramer Kenneth L
Vestibular Genetics Laboratory, Boys Town National Research Hospital, Omaha, Nebraska.
Dev Dyn. 2015 Mar;244(3):239-53. doi: 10.1002/dvdy.24195. Epub 2014 Oct 18.
Otoconia are bio-crystals that couple mechanic forces to the sensory hair cells in the utricle and saccule, a process essential for us to sense linear acceleration and gravity for the purpose of maintaining bodily balance. In fish, structurally similar bio-crystals called otoliths mediate both balance and hearing. Otoconia abnormalities are common and can cause vertigo and imbalance in humans. However, the molecular etiology of these illnesses is unknown, as investigators have only begun to identify genes important for otoconia formation in recent years.
To date, in-depth studies of selected mouse otoconial proteins have been performed, and about 75 zebrafish genes have been identified to be important for otolith development.
This review will summarize recent findings as well as compare otoconia and otolith development. It will provide an updated brief review of otoconial proteins along with an overview of the cells and cellular processes involved. While continued efforts are needed to thoroughly understand the molecular mechanisms underlying otoconia and otolith development, it is clear that the process involves a series of temporally and spatially specific events that are tightly coordinated by numerous proteins. Such knowledge will serve as the foundation to uncover the molecular causes of human otoconia-related disorders.
耳石是一种生物晶体,可将机械力传递给椭圆囊和球囊中的感觉毛细胞,这一过程对我们感知线性加速度和重力以维持身体平衡至关重要。在鱼类中,结构类似的生物晶体耳石同时介导平衡和听觉。耳石异常很常见,可导致人类眩晕和失衡。然而,这些疾病的分子病因尚不清楚,因为研究人员直到近年来才开始确定对耳石形成重要的基因。
迄今为止,已对选定的小鼠耳石蛋白进行了深入研究,并且已确定约75个斑马鱼基因对耳石发育很重要。
本综述将总结近期的研究发现,并比较耳石和耳石的发育。它将提供耳石蛋白的最新简要综述以及所涉及的细胞和细胞过程的概述。虽然需要持续努力以彻底了解耳石和耳石发育的分子机制,但很明显该过程涉及一系列在时间和空间上特定的事件,这些事件由众多蛋白质紧密协调。这些知识将成为揭示人类耳石相关疾病分子病因的基础。