Deletion of C1ql1 Causes Hearing Loss and Abnormal Auditory Nerve Fibers in the Mouse Cochlea.
Deletion of C1ql1 Causes Hearing Loss and Abnormal Auditory Nerve Fibers in the Mouse Cochlea.
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C1ql1 缺失导致小鼠耳蜗听力损失和听觉神经纤维异常
DOI：
10.3389/fncel.2021.713651
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发表时间：
2021
影响因子：
5.3
通讯作者：
Gong S
中科院分区：
文献类型：
--
作者：
Qi Y;Xiong W;Yu S;Du Z;Qu T;He L;Wei W;Zhang L;Liu K;Li Y;He DZ;Gong S
补体C1 q样1（C1 QL 1）是C1 Q相关蛋白的分泌成分，在中枢神经系统突触成熟、调节和维持中发挥重要作用。C1 ql 1在成年人耳蜗内毛细胞和外毛细胞中表达，并在外毛细胞中优先表达。我们产生了C1 ql 1基因敲除小鼠来研究C1 QL 1在听觉外周的作用。C1 ql 1基因敲除小鼠表现出进行性听力损失，听性脑干反应和畸变产物耳声发射阈值升高。共聚焦显微镜显示支配内毛细胞和外毛细胞的神经纤维数量显著减少。然而，螺旋神经节神经元在电子显微镜下似乎是正常的。C1 ql 1缺失不影响IHC发育和存活。电压钳记录和免疫细胞化学结合共聚焦显微镜显示，C1 ql 1-null IHC显示突触前蛋白和突触囊泡释放没有显着减少。这与KO小鼠中的显著OHC损失相反。我们的研究表明，C1 ql 1是必不可少的毛细胞神经支配和OHC生存的发展。但在IHC中突触前机制的成熟并不依赖于C1 QL 1。
Complement C1q Like 1 (C1QL1), a secreted component of C1Q-related protein, is known to play an important role in synaptic maturation, regulation, and maintenance in the central nervous system. C1ql1 is expressed in adult cochlear inner and outer hair cells (IHCs and OHCs) with preferential expression in OHCs. We generated C1ql1 null mice to examine the role of C1QL1 in the auditory periphery. C1ql1-null mice exhibited progressive hearing loss with elevated thresholds of auditory brainstem response and distortion product otoacoustic emission. Confocal microscopy showed that the number of nerve fibers innervating both IHCs and OHCs was significantly reduced. However, spiral ganglion neurons appeared to be normal under electron microscopy. IHC development and survival were not affected by deletion of C1ql1. Voltage-clamp recording and immunocytochmistry combined with confocal microscopy showed C1ql1-null IHCs showed no significant reduction of pre-synaptic proteins and synaptic vesicle release. This is in contrast to significant OHC loss in the KO mice. Our study suggests that C1ql1 is essential for development of hair cell innervation and OHC survival. But maturation of presynaptic machinery in IHCs does not depend on C1QL1.
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影响因子：
8.8
作者：
Ranum, Paul T.;Goodwin, Alexander T.;Smith, Richard J. H.
通讯作者：
Smith, Richard J. H.
细胞特异性转录组分析表明，成体支柱细胞和 Deiters 细胞表达耳蜗毛细胞特化的基因编码机制
影响因子：
4.8
作者：
Liu H;Chen L;Giffen KP;Stringham ST;Li Y;Judge PD;Beisel KW;He DZZ
通讯作者：
He DZZ
影响因子：
8.8
作者：
Sigoillot, Severine M.;Iyer, Keerthana;Selimi, Fekrije
通讯作者：
Selimi, Fekrije
影响因子：
2.7
作者：
Shimono, Chisei;Manabe, Ri-ichiroh;Sekiguchi, Kiyotoshi
通讯作者：
Sekiguchi, Kiyotoshi
影响因子：
3.4
作者：
Maison S;Liberman LD;Liberman MC
通讯作者：
Liberman MC