Using Drosophila to study mechanisms of hereditary hearing loss.

Johnston's organ – the hearing organ of Drosophila – has a very different structure and morphology to that of the hearing organs of vertebrates. Nevertheless, it is becoming clear that vertebrate and invertebrate auditory organs share many physiological, molecular and genetic similarities. Here, we compare the molecular and cellular features of hearing organs in Drosophila with those of vertebrates, and discuss recent evidence concerning the functional conservation of Usher proteins between flies and mammals. Mutations in Usher genes cause Usher syndrome, the leading cause of human deafness and blindness. In Drosophila, some Usher syndrome proteins appear to physically interact in protein complexes that are similar to those described in mammals. This functional conservation highlights a rational role for Drosophila as a model for studying hearing, and for investigating the evolution of auditory organs, with the aim of advancing our understanding of the genes that regulate human hearing and the pathogenic mechanisms that lead to deafness. Summary: This Review discusses the similarities and differences between hearing in mammals and the fruit fly Drosophila, and describes how recent technological developments allow Drosophila to be used as a model to understand the function of human deafness genes.

约翰斯顿氏器——果蝇的听觉器官——其结构和形态与脊椎动物的听觉器官有很大不同。然而，越来越明显的是，脊椎动物和无脊椎动物的听觉器官在生理、分子和遗传方面有许多相似之处。在此，我们比较了果蝇听觉器官与脊椎动物听觉器官的分子和细胞特征，并讨论了有关果蝇和哺乳动物之间Usher蛋白功能保守性的最新证据。Usher基因的突变会导致Usher综合征，这是人类耳聋和失明的主要原因。在果蝇中，一些Usher综合征蛋白似乎在蛋白质复合物中发生物理相互作用，这些复合物与在哺乳动物中所描述的相似。这种功能保守性凸显了果蝇作为研究听觉以及探究听觉器官进化的模型的合理作用，目的是增进我们对调节人类听觉的基因以及导致耳聋的致病机制的理解。 总结：这篇综述讨论了哺乳动物和果蝇的听觉之间的异同，并描述了近期的技术发展如何使果蝇能够被用作一种模型来理解人类耳聋基因的功能。