Transmission Disrupted: Modeling Auditory Synaptopathy in Zebrafish.
Transmission Disrupted: Modeling Auditory Synaptopathy in Zebrafish.
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DOI：
10.3389/fcell.2018.00114
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发表时间：
2018
影响因子：
5.5
通讯作者：
Sheets L
中科院分区：
文献类型：
--
作者：
Kindt KS;Sheets L
感音神经性听力损失是人类最常见的听力损失形式，是由毛细胞、声音感觉受体或支配毛细胞的神经元功能障碍引起的。当毛细胞能够检测声音但无法在毛细胞突触处传输声音刺激时，就会发生一种特殊类型的感音神经性听力损失，称为听觉突触病。听觉突触病可能源于专门破坏毛细胞突触功能的基因改变。此外，环境因素（例如噪音暴露）可以使毛细胞保持完整，但会导致毛细胞突触丧失，并代表一种获得性的听觉突触病。斑马鱼模型已成为研究毛细胞功能，特别是毛细胞突触病的有价值的系统。在这篇综述中，我们描述了为研究斑马鱼毛细胞突触而开发的实验工具。我们讨论斑马鱼遗传学如何帮助识别和定义对人类听力至关重要的毛细胞突触蛋白的作用，并强调斑马鱼研究如何有助于我们理解毛细胞突触的形成和功能。此外，我们还讨论了使用噪声暴露或噪声引起的斑马鱼兴奋性毒性的药理学模拟来定义噪声引起的毛细胞损伤和突触损失的细胞机制。最后，我们强调未来对斑马鱼的研究如何能够增强我们对遗传性和获得性听觉突触病中突触丧失的病理过程的理解。这些知识对于开发保护或修复听觉突触接触的疗法至关重要。
Sensorineural hearing loss is the most common form of hearing loss in humans, and results from either dysfunction in hair cells, the sensory receptors of sound, or the neurons that innervate hair cells. A specific type of sensorineural hearing loss, referred to as auditory synaptopathy, occurs when hair cells are able to detect sound but fail to transmit sound stimuli at the hair-cell synapse. Auditory synaptopathy can originate from genetic alterations that specifically disrupt hair-cell synapse function. Additionally, environmental factors such as noise exposure can leave hair cells intact but result in loss of hair-cell synapses, and represent an acquired form of auditory synaptopathy. The zebrafish model has emerged as a valuable system for studies of hair-cell function, and specifically hair-cell synaptopathy. In this review, we describe the experimental tools that have been developed to study hair-cell synapses in zebrafish. We discuss how zebrafish genetics has helped identify and define the roles of hair-cell synaptic proteins crucial for hearing in humans, and highlight how studies in zebrafish have contributed to our understanding of hair-cell synapse formation and function. In addition, we also discuss work that has used noise exposure or pharmacological mimic of noise-induced excitotoxicity in zebrafish to define cellular mechanisms underlying noise-induced hair-cell damage and synapse loss. Lastly, we highlight how future studies in zebrafish could enhance our understanding of the pathological processes underlying synapse loss in both genetic and acquired auditory synaptopathy. This knowledge is critical in order to develop therapies that protect or repair auditory synaptic contacts.
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影响因子：
16.2
作者：
Frank, Thomas;Rutherford, Mark A.;Strenzke, Nicola;Neef, Andreas;Pangrsic, Tina;Khimich, Darina;Fetjova, Anna;Gundelfinger, Eckart D.;Liberman, M. Charles;Harke, Benjamin;Bryan, Keith E.;Lee, Amy;Egner, Alexander;Riedel, Dietmar;Moser, Tobias
通讯作者：
Moser, Tobias
影响因子：
3
作者：
Bharadwaj HM;Verhulst S;Shaheen L;Liberman MC;Shinn-Cunningham BG
通讯作者：
Shinn-Cunningham BG
影响因子：
10.5
作者：
Dow E;Siletti K;Hudspeth AJ
通讯作者：
Hudspeth AJ
影响因子：
5.3
作者：
Dinh CT;Goncalves S;Bas E;Van De Water TR;Zine A
通讯作者：
Zine A
影响因子：
1.4
作者：
BOHNE, BA
通讯作者：
BOHNE, BA