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Deletion of C1ql1 Causes Hearing Loss and Abnormal Auditory Nerve Fibers in the Mouse Cochlea.

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机构： [1]Department of Otolaryngology Head and Neck Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, China, [2]Department of Otology, Sheng Jing Hospital, China Medical University, Shenyang, China, [3]Department of Otolaryngology Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing, China, [4]Department of Biomedical Sciences, Creighton University, Omaha, NE, United States

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关键词： C1QL1 hair cells innervation hearing loss mice

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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.Copyright © 2021 Qi, Xiong, Yu, Du, Qu, He, Wei, Zhang, Liu, Li, He and Gong.

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出版当年[2020]版：

大类 | 2 区医学

小类 | 3 区神经科学

最新[2025]版：

大类 | 3 区医学

小类 | 3 区神经科学

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出版当年[2019]版：

Q2 NEUROSCIENCES

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Q2 NEUROSCIENCES

影响因子： 4 最新[2024版] 4.9 最新五年平均 3.921 出版当年[2019版] 4.438 出版当年五年平均 3.9 出版前一年[2018版] 5.505 出版后一年[2020版]

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第一作者机构： [1]Department of Otolaryngology Head and Neck Surgery, Beijing Friendship Hospital, Capital Medical University, Beijing, China,

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