Vlgr1在听觉系统中功能的研究进展
首发时间:2012-11-02
摘要:G蛋白偶联受体(GPCR)是一类七跨膜蛋白,是最大的蛋白超家族之一,其介导的信号传导参与多种生理过程。G蛋白偶联受体在视觉、嗅觉、味觉等感觉系统中发挥重要作用;事实上,视觉受体、嗅觉受体以及部分味觉受体都是G蛋白偶联受体。但迄今为止我们对G蛋白偶联受体在听觉系统中的作用所知甚少。目前已知的在听觉系统表达的GPCR除AChR及Adenosine受体外,还有Vlgr1和Celsr1等。Vlgr1是目前已知最大的G蛋白偶联受体,其mRNA长达19kb,编码6300多个氨基酸。Vlgr1蛋白在毛细胞听纤毛的根部表达,参与听纤毛踝连接(ankle link)的形成。Vlgr1基因突变可以引起Usher综合症(感觉神经性耳聋和色素性视网膜炎)以及癫痫。尽管Vlgr1研究已经取得了很多的进展,但Vlgr1蛋白在毛细胞中的作用机制,介导信号传导的过程和由基因突变所引发疾病的机制仍需要更进一步的研究。
关键词: 神经生物学 Vlgr1 G蛋白偶联受体 听觉传导 Usher综合征
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The Very Large G protein-coupled Receptor (Vlgr1) in auditory systems
Abstract:G protein-coupled receptors (GPCR) are seven trans-membrane helical proteins, which sense the extracellular stimuli and regulate cellular responses. The genomic projects have identified GPCRs as one of the largest protein super-families. GPCRs play important roles in visual, olfactory and gustatory systems; in fact, visual, olfactory and some gustatory receptors are GPCRs. But the role of GPCRs in auditory system is less understood. Known GPCRs expressed in auditory systems include AchR, Adenosin receptors, Celsr1, and Vlgr1. Vlgr1 is the largest GPCR identified to date; its mRNA spans 19kb and encodes 6300 amino acids. Vlgr1 is a core component of ankle-link complex in inner ear hair cells. Over the past 10 or so years, human genetics data suggested that Vlgr1 mutations cause Usher syndromes and seizures. Although significant progresses have been made, the details of Vlgr1's function in hair cells, its signaling cascade, and the mechanisms underlying causative effects of Vlgr1 mutations in human diseases remain elusive and ask for further investigation.
Keywords: Neurobiology Vlgr1 GPCR Hearing transduction Usher syndrome
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