Mitochondrial alterations in the cochlea of Cdk5rap1‐knockout mice with age‐related hearing loss

Previous studies have revealed that age‐related hearing loss (AHL) in Cdk5 regulatory subunit‐associated protein 1 (Cdk5rap1)‐knockout mice is associated with pathology in the cochlea. Here, we aimed to identify mitochondrial alterations in the cochlea of Cdk5rap1‐knockout mice with AHL. Mitochondri...

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Main Authors: Toru Miwa, Tatsuya Katsuno, Fan‐Yan Wei, Kazuhito Tomizawa
Format: Article
Language:English
Published: Wiley 2023-07-01
Series:FEBS Open Bio
Subjects:
Online Access:https://doi.org/10.1002/2211-5463.13655
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author Toru Miwa
Tatsuya Katsuno
Fan‐Yan Wei
Kazuhito Tomizawa
author_facet Toru Miwa
Tatsuya Katsuno
Fan‐Yan Wei
Kazuhito Tomizawa
author_sort Toru Miwa
collection DOAJ
description Previous studies have revealed that age‐related hearing loss (AHL) in Cdk5 regulatory subunit‐associated protein 1 (Cdk5rap1)‐knockout mice is associated with pathology in the cochlea. Here, we aimed to identify mitochondrial alterations in the cochlea of Cdk5rap1‐knockout mice with AHL. Mitochondria in the spiral ganglion neurons (SGNs) and hair cells (HCs) were normal despite senescence; however, the mitochondria of types I, II, and IV spiral ligament fibrocytes were ballooned, damaged, and ballooned, respectively, in the stria vascularis. Our results suggest that the accumulation of dysfunctional mitochondria in the lateral wall, rather than the loss of HCs and SGNs, leads to the onset of AHL. Our results provide valuable information regarding the underlying mechanisms of AHL and the relationship between aberrant tRNA modification‐induced hearing loss and mitochondrial dysfunction.
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spelling doaj.art-3ed5431d3e4944b88ffb410a89c829662023-07-03T07:24:35ZengWileyFEBS Open Bio2211-54632023-07-011371365137410.1002/2211-5463.13655Mitochondrial alterations in the cochlea of Cdk5rap1‐knockout mice with age‐related hearing lossToru Miwa0Tatsuya Katsuno1Fan‐Yan Wei2Kazuhito Tomizawa3Department of Otolaryngology‐Head and Neck Surgery, Graduate School of Medicine Kyoto University JapanDepartment of Otolaryngology‐Head and Neck Surgery Osaka Metropolitan University JapanDepartment of Molecular Physiology, Faculty of Life Sciences Kumamoto University JapanDepartment of Molecular Physiology, Faculty of Life Sciences Kumamoto University JapanPrevious studies have revealed that age‐related hearing loss (AHL) in Cdk5 regulatory subunit‐associated protein 1 (Cdk5rap1)‐knockout mice is associated with pathology in the cochlea. Here, we aimed to identify mitochondrial alterations in the cochlea of Cdk5rap1‐knockout mice with AHL. Mitochondria in the spiral ganglion neurons (SGNs) and hair cells (HCs) were normal despite senescence; however, the mitochondria of types I, II, and IV spiral ligament fibrocytes were ballooned, damaged, and ballooned, respectively, in the stria vascularis. Our results suggest that the accumulation of dysfunctional mitochondria in the lateral wall, rather than the loss of HCs and SGNs, leads to the onset of AHL. Our results provide valuable information regarding the underlying mechanisms of AHL and the relationship between aberrant tRNA modification‐induced hearing loss and mitochondrial dysfunction.https://doi.org/10.1002/2211-5463.13655age‐related hearing lossCdk5rap1microstructural findingsmitochondriamitochondrial tRNAtransmission electron microscopy
spellingShingle Toru Miwa
Tatsuya Katsuno
Fan‐Yan Wei
Kazuhito Tomizawa
Mitochondrial alterations in the cochlea of Cdk5rap1‐knockout mice with age‐related hearing loss
FEBS Open Bio
age‐related hearing loss
Cdk5rap1
microstructural findings
mitochondria
mitochondrial tRNA
transmission electron microscopy
title Mitochondrial alterations in the cochlea of Cdk5rap1‐knockout mice with age‐related hearing loss
title_full Mitochondrial alterations in the cochlea of Cdk5rap1‐knockout mice with age‐related hearing loss
title_fullStr Mitochondrial alterations in the cochlea of Cdk5rap1‐knockout mice with age‐related hearing loss
title_full_unstemmed Mitochondrial alterations in the cochlea of Cdk5rap1‐knockout mice with age‐related hearing loss
title_short Mitochondrial alterations in the cochlea of Cdk5rap1‐knockout mice with age‐related hearing loss
title_sort mitochondrial alterations in the cochlea of cdk5rap1 knockout mice with age related hearing loss
topic age‐related hearing loss
Cdk5rap1
microstructural findings
mitochondria
mitochondrial tRNA
transmission electron microscopy
url https://doi.org/10.1002/2211-5463.13655
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AT tatsuyakatsuno mitochondrialalterationsinthecochleaofcdk5rap1knockoutmicewithagerelatedhearingloss
AT fanyanwei mitochondrialalterationsinthecochleaofcdk5rap1knockoutmicewithagerelatedhearingloss
AT kazuhitotomizawa mitochondrialalterationsinthecochleaofcdk5rap1knockoutmicewithagerelatedhearingloss