The genetics of hair-cell function in zebrafish.
The genetics of hair-cell function in zebrafish.
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DOI：
10.1080/01677063.2017.1342246
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发表时间：
2017-09
影响因子：
1.9
通讯作者：
Nicolson T
中科院分区：
文献类型：
--
作者：
Nicolson T
我们的耳朵是非凡的感觉器官，提供重要的平衡感和听觉。内耳的复杂结构，或称“内耳”，沿着各种神经上皮细胞，已经进化到能够以令人印象深刻的灵敏度检测头部运动和声音。问题是内耳非常容易受到遗传损伤和环境损害的影响。根据美国国立卫生研究院的估计，听力损失是最常见的遗传性或后天性感觉神经性疾病之一。要了解耳聋和平衡障碍的原因，必须了解内耳的基本生物学，特别是感觉受体的内部工作。这些被称为毛细胞的受体特别容易受到基因突变的影响-超过24个基因与人类这种细胞类型的缺陷有关。在过去的十年中，在利用脊椎动物模型研究毛细胞功能的分子基础方面取得了很大的进展。由于内耳的透明性和可用的遗传工具，斑马鱼已成为研究耳聋和前庭功能障碍的越来越受欢迎的动物模型。针对听力和平衡缺陷的幼虫突变筛选在发现关键成分方面取得了丰硕成果，其中许多成分与人类耳聋有关。这篇评论将集中在斑马鱼毛细胞功能所需的基因，特别强调机械转导。此外，将讨论可用于表征斑马鱼毛细胞突变体的新工具的产生。
Our ears are remarkable sensory organs, providing the important senses of balance and hearing. The complex structure of the inner ear, or ‘labyrinth’, along with the assorted neuroepithelia, has evolved to detect head movements and sounds with impressive sensitivity. The rub is that the inner ear is highly vulnerable to genetic lesions and environmental insults. According to National Institute of Health estimates, hearing loss is one of the most commonly inherited or acquired sensorineural diseases. To understand the causes of deafness and balance disorders, it is imperative to understand the underlying biology of the inner ear, especially the inner workings of the sensory receptors. These receptors, which are termed hair cells, are particularly susceptible to genetic mutations--more than two dozen genes are associated with defects in this cell type in humans. Over the past decade, a substantial amount of progress has been made in working out the molecular basis of hair-cell function using vertebrate animal models. Given the transparency of the inner ear and the genetic tools that are available, zebrafish have become an increasingly popular animal model for the study of deafness and vestibular dysfunction. Mutagenesis screens for larval defects in hearing and balance have been fruitful in finding key components, many of which have been implicated in human deafness. This review will focus on the genes that are required for hair-cell function in zebrafish, with a particular emphasis on mechanotransduction. In addition, the generation of new tools available for the characterization of zebrafish hair-cell mutants will be discussed.
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影响因子：
4.4
作者：
Daniele LL;Emran F;Lobo GP;Gaivin RJ;Perkins BD
通讯作者：
Perkins BD
DOI：
10.1242/jeb.082362
发表时间：
2013-07-01
期刊：
The Journal of experimental biology
影响因子：
--
作者：
Baker CV;Modrell MS;Gillis JA
通讯作者：
Gillis JA
DOI：
10.1083/jcb.15.1.19
发表时间：
1962-10
期刊：
The Journal of cell biology
影响因子：
--
作者：
FLOCK A;WERSALL J
通讯作者：
WERSALL J
DOI：
10.1002/neu.10098
发表时间：
2002-11-05
期刊：
JOURNAL OF NEUROBIOLOGY
影响因子：
--
作者：
Fritzsch, B;Beisel, KW;Reichardt, LF
通讯作者：
Reichardt, LF
影响因子：
5.3
作者：
Corey, David P.;Holt, Jeffrey R.
通讯作者：
Holt, Jeffrey R.