The sound and the fly: Drosophila in biomedical research on human hearing

M. Capovilla
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Abstract

Deep biochemical and cellular similarities exist between Drosophila and vertebrates in the development and function of all organs. Despite their morphological differences, it is considered ever more likely that the hearing sensory cells have a common evolutionary origin and that consequently the molecular mechanisms that govern their functions are at least partially conserved. Thanks to transgenesis techniques and to the rich collection of mutant stocks well characterized from genetic, molecular and physiological points of view, Drosophila provides the means to test in vivo vertebrate gene functions in known metabolic and cellular pathways. In addition, the search for sequence homologies between human and Drosophila genes has allowed the identification of many disease genes in the human auditory system. These results indicate that Drosophila is a good model for the study of the genes that cause deafness in humans, and offers the possibility of using the numerous and sophisticated genetic tools of the insect to decipher how mechanical signals are elaborated by the auditory system. In this review, contributions to biomedical research from studies on Drosophila mutants are briefly summarized following a concise account on the fruitfly life cycle, tools and available resources. Particular attention is paid to studies on the genetic control of hearing mechanisms in the insect. The acquired knowledge will help confront some vertebrate hearing diseases on a molecular basis.
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声音与苍蝇:人类听觉生物医学研究中的果蝇
果蝇和脊椎动物在所有器官的发育和功能上存在着深刻的生化和细胞相似性。尽管它们在形态上存在差异,但更有可能的是,听觉感觉细胞有一个共同的进化起源,因此,控制它们功能的分子机制至少部分是保守的。由于转基因技术和从遗传、分子和生理角度充分表征的丰富突变体,果蝇提供了在已知代谢和细胞途径中测试体内脊椎动物基因功能的手段。此外,寻找人类和果蝇基因之间的序列同源性使得人类听觉系统中许多疾病基因的鉴定成为可能。这些结果表明,果蝇是研究导致人类耳聋的基因的一个很好的模型,并提供了利用昆虫大量和复杂的遗传工具来破译听觉系统如何阐述机械信号的可能性。在这篇综述中,简要总结了果蝇突变体研究对生物医学研究的贡献,并简要介绍了果蝇的生命周期、工具和可用资源。特别关注昆虫听觉机制的遗传控制研究。所获得的知识将有助于在分子基础上对抗一些脊椎动物听力疾病。
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Referees Morphological and functional structure of the inner ear: Its relation to Ménière's disease Medical therapy in Ménière's disease Simon Dafydd Glyn Stephens, Professor of Audiological Medicine Ménière's disorder: A short history
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