Buniello, A., Ingham, N.J., Lewis, M.A. et al. (12 more authors) (2016) Wbp2 is required for normal glutamatergic synapses in the cochlea and is crucial for hearing. EMBO Molecular Medicine, 8 (3). pp. 191-207. ISSN 1757-4676
Abstract
WBP2 encodes the WW domain-binding protein 2 that acts as a transcriptional coactivator for estrogen receptor a (ESR1) and progesterone receptor (PGR). We reported that the loss of Wbp2 expression leads to progressive high-frequency hearing loss in mouse, as well as in two deaf children, each carrying two different variants in the WBP2 gene. The earliest abnormality we detect in Wbp2-deficient mice is a primary defect at inner hair cell afferent synapses. This study defines a new gene involved in the molecular pathway linking hearing impairment to hormonal signalling and provides new therapeutic targets.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Copyright, Publisher and Additional Information: | © 2016 The Authors . This is an open access article under the terms of the Creative Commons Attribution 4.0 License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. |
Keywords: | glutamate excitotoxicity; hearing impairment; hormonal signalling; ribbon synapses; transcriptional coactivator |
Dates: |
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Institution: | The University of Sheffield |
Academic Units: | The University of Sheffield > Faculty of Science (Sheffield) > School of Biosciences (Sheffield) > Department of Biomedical Science (Sheffield) |
Depositing User: | Symplectic Sheffield |
Date Deposited: | 09 Jan 2017 16:47 |
Last Modified: | 09 Jan 2017 16:47 |
Published Version: | http://doi.org/10.15252/emmm.201505523 |
Status: | Published |
Publisher: | Wiley Open Access |
Refereed: | Yes |
Identification Number: | 10.15252/emmm.201505523 |
Related URLs: | |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:108200 |