Type IX collagen knock-out mouse shows progressive hearing loss
Type IX collagen knock-out mouse shows progressive hearing loss
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DOI：
10.1016/j.neures.2004.12.001
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发表时间：
2005-03-01
影响因子：
2.9
通讯作者：
Usami, S
中科院分区：
文献类型：
--
作者：
Suzuki, N;Asamura, K;Usami, S
IX型胶原蛋白与11型、V型和XI型胶原蛋白一起是柯蒂氏器盖膜的重要成分之一。为了证实 IX 型胶原蛋白对正常听力的重要性，我们评估了 IX 型胶原蛋白敲除小鼠的详细形态学和电生理学特征，该小鼠最近被报道为耳聋模型。通过听觉脑干反应（ABR）评估，基因敲除小鼠出现进行性听力损失。在光镜水平上，发现基因敲除小鼠的盖膜形状异常。这些形态变化始于基部转，并逐渐向顶端转发展。电子显微镜证实胶原原纤维的组织受到干扰。这些结果表明 IX 型胶原蛋白基因突变可能导致盖膜中胶原纤维的完整性异常。(C) 2004 Elsevier Ireland Ltd 和日本神经科学学会。版权所有。
Type IX collagen is one of the important components, together with type 11, V, and XI collagens, in the tectorial membrane of the organ of Corti. To confirm the significance of type IX collagen for normal hearing, we assessed the detailed morphological and electrophysiological features of type IX collagen knock-out mice, which have recently been reported as a deafness model. Through assessment by auditory brainstem response (ABR), knock-out mice were shown to have progressive hearing loss. At the light microscopic level, the tectorial membrane of knock-out mice was found to be abnormal in shape. These morphological changes started in the basal turn and were progressive toward the apical turn. Electron microscopy confirmed disturbance of organization of the collagen fibrils. These results suggest that mutations in type IX collagen genes may lead to abnormal integrity of collagen fibers in the tectorial membrane.(C) 2004 Elsevier Ireland Ltd and the Japan Neuroscience Society. All rights reserved.