Mitochondrial alterations in the cochlea of Cdk5rap 1-knockout mice with age-related hearing loss

被引：0

作者：

Miwa, Toru ^{[1
,2
,5
]}

Katsuno, Tatsuya ^{[2
]}

Wei, Fan-Yan ^{[3
,4
]}

Tomizawa, Kazuhito ^{[3
]}

机构：

[1] Kyoto Univ, Grad Sch Med, Dept Otolaryngol Head & Neck Surg, Kyoto, Japan

[2] Osaka Metropolitan Univ, Dept Otolaryngol Head & Neck Surg, Osaka, Japan

[3] Kumamoto Univ, Fac Life Sci, Dept Mol Physiol, Kumamoto, Japan

[4] Tohoku Univ, Inst Dev Aging & Canc, Dept Mod Biol & Med, Sendai, Japan

[5] Osaka Metropolitan Univ, Dept Otolaryngol Head & Neck Surg, 1-4-3 Asahi Machi,Abeno Ku, Osaka 5458585, Japan

来源：

FEBS OPEN BIO | 2023年 / 13卷 / 07期

关键词：

age-related hearing loss; Cdk5rap1; microstructural findings; mitochondria; mitochondrial tRNA; transmission electron microscopy; DEGENERATION; DYSFUNCTION; MUTATION; DNA;

D O I：

10.1002/2211-5463.13655

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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.

引用

页码：1365 / 1374

页数：10

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