Requena, T;
Keder, A;
zur Lage, P;
Albert, JT;
Jarman, AP;
(2022)
A Drosophila model for Meniere's disease: Dystrobrevin is required for support cell function in hearing and proprioception.
Frontiers in Cell and Developmental Biology
, 10
, Article 1015651. 10.3389/fcell.2022.1015651.
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A iDrosophilai model for Menieres disease Dystrobrevin is required for support cell function in hearing and proprioception.pdf - Published Version Download (3MB) | Preview |
Abstract
Meniere's disease (MD) is an inner ear disorder characterised by recurrent vertigo attacks associated with sensorineural hearing loss and tinnitus. Evidence from epidemiology and Whole Exome Sequencing (WES) suggests a genetic susceptibility involving multiple genes, including α-Dystrobrevin (DTNA). Here we investigate a Drosophila model. We show that mutation, or knockdown, of the DTNA orthologue in Drosophila, Dystrobrevin (Dyb), results in defective proprioception and impaired function of Johnston's Organ (JO), the fly's equivalent of the inner ear. Dyb and another component of the dystrophin-glycoprotein complex (DGC), Dystrophin (Dys), are expressed in support cells within JO. Their specific locations suggest that they form part of support cell contacts, thereby helping to maintain the integrity of the hemolymph-neuron diffusion barrier, which is equivalent to a blood-brain barrier. These results have important implications for the human condition, and notably, we note that DTNA is expressed in equivalent cells of the mammalian inner ear.
| Type: | Article |
|---|---|
| Title: | A Drosophila model for Meniere's disease: Dystrobrevin is required for support cell function in hearing and proprioception |
| Location: | Switzerland |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.3389/fcell.2022.1015651 |
| Publisher version: | https://doi.org/10.3389/fcell.2022.1015651 |
| Language: | English |
| Additional information: | © 2022 Requena, Keder, zur Lage, Albert and Jarman. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
| Keywords: | Science & Technology, Life Sciences & Biomedicine, Cell Biology, Developmental Biology, Meniere's disease, Drosophila, dystrobrevin, animal model, dystrophin, hearing, DYSTROPHIN-GLYCOPROTEIN COMPLEX, SYNTHASE INOS/NOS-II, NITRIC-OXIDE, ALPHA-DYSTROGLYCAN, GENETIC-HETEROGENEITY, DIAGNOSTIC-CRITERIA, MUSCULAR-DYSTROPHY, COCHLEAR FUNCTION, NERVOUS-SYSTEM, HAIR-CELLS |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > The Ear Institute |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10162214 |
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