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Knockout of Pannexin-1 Induces Hearing Loss
Mutations of gap junction connexin genes induce a high incidence of nonsyndromic hearing loss. Pannexin genes also encode gap junctional proteins in vertebrates. Recent studies demonstrated that Pannexin-1 (Panx1) deficiency in mice and mutation in humans are also associated with hearing loss. So fa...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5983795/ https://www.ncbi.nlm.nih.gov/pubmed/29710868 http://dx.doi.org/10.3390/ijms19051332 |
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author | Chen, Jin Liang, Chun Zong, Liang Zhu, Yan Zhao, Hong-Bo |
author_facet | Chen, Jin Liang, Chun Zong, Liang Zhu, Yan Zhao, Hong-Bo |
author_sort | Chen, Jin |
collection | PubMed |
description | Mutations of gap junction connexin genes induce a high incidence of nonsyndromic hearing loss. Pannexin genes also encode gap junctional proteins in vertebrates. Recent studies demonstrated that Pannexin-1 (Panx1) deficiency in mice and mutation in humans are also associated with hearing loss. So far, several Panx1 knockout (KO) mouse lines were established. In general, these Panx1 KO mouse lines demonstrate consistent phenotypes in most aspects, including hearing loss. However, a recent study reported that a Panx1 KO mouse line, which was created by Genentech Inc., had no hearing loss as measured by the auditory brainstem response (ABR) threshold at low-frequency range (<24 kHz). Here, we used multiple auditory function tests and re-examined hearing function in the Genentech Panx1 (Gen-Panx1) KO mouse. We found that ABR thresholds in the Gen-Panx1 KO mouse were significantly increased, in particular, in the high-frequency region. Moreover, consistent with the increase in ABR threshold, distortion product otoacoustic emission (DPOAE) and cochlear microphonics (CM), which reflect active cochlear amplification and auditory receptor current, respectively, were significantly reduced. These data demonstrated that the Gen-Panx1 KO mouse has hearing loss and further confirmed that Panx1 deficiency can cause deafness. |
format | Online Article Text |
id | pubmed-5983795 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-59837952018-06-05 Knockout of Pannexin-1 Induces Hearing Loss Chen, Jin Liang, Chun Zong, Liang Zhu, Yan Zhao, Hong-Bo Int J Mol Sci Article Mutations of gap junction connexin genes induce a high incidence of nonsyndromic hearing loss. Pannexin genes also encode gap junctional proteins in vertebrates. Recent studies demonstrated that Pannexin-1 (Panx1) deficiency in mice and mutation in humans are also associated with hearing loss. So far, several Panx1 knockout (KO) mouse lines were established. In general, these Panx1 KO mouse lines demonstrate consistent phenotypes in most aspects, including hearing loss. However, a recent study reported that a Panx1 KO mouse line, which was created by Genentech Inc., had no hearing loss as measured by the auditory brainstem response (ABR) threshold at low-frequency range (<24 kHz). Here, we used multiple auditory function tests and re-examined hearing function in the Genentech Panx1 (Gen-Panx1) KO mouse. We found that ABR thresholds in the Gen-Panx1 KO mouse were significantly increased, in particular, in the high-frequency region. Moreover, consistent with the increase in ABR threshold, distortion product otoacoustic emission (DPOAE) and cochlear microphonics (CM), which reflect active cochlear amplification and auditory receptor current, respectively, were significantly reduced. These data demonstrated that the Gen-Panx1 KO mouse has hearing loss and further confirmed that Panx1 deficiency can cause deafness. MDPI 2018-04-30 /pmc/articles/PMC5983795/ /pubmed/29710868 http://dx.doi.org/10.3390/ijms19051332 Text en © 2018 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Chen, Jin Liang, Chun Zong, Liang Zhu, Yan Zhao, Hong-Bo Knockout of Pannexin-1 Induces Hearing Loss |
title | Knockout of Pannexin-1 Induces Hearing Loss |
title_full | Knockout of Pannexin-1 Induces Hearing Loss |
title_fullStr | Knockout of Pannexin-1 Induces Hearing Loss |
title_full_unstemmed | Knockout of Pannexin-1 Induces Hearing Loss |
title_short | Knockout of Pannexin-1 Induces Hearing Loss |
title_sort | knockout of pannexin-1 induces hearing loss |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5983795/ https://www.ncbi.nlm.nih.gov/pubmed/29710868 http://dx.doi.org/10.3390/ijms19051332 |
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