Expression of cochlin in the vestibular organ of rats
Expression of cochlin in the vestibular organ of rats
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DOI：
10.1159/000089404
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发表时间：
2005-01-01
期刊：
影响因子：
--
通讯作者：
Yagi, T
中科院分区：
文献类型：
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作者：
Ikezono, T;Shindo, S;Yagi, T
在常染色体显性遗传性感音神经性耳聋(DFNA9)中，COCH基因突变编码内耳细胞外基质的主要成分Cochlin。Cochlin占内耳蛋白质的70%，Cochlin亚型可分为p63s、p44s和p40s三个亚类。一些DFNA9患者的症状与梅尼埃病的症状一致。在这里，我们报告了使用识别所有三种异构体的异构体特异性抗体在大鼠前庭器官中表达Cochlin。Cochlin高表达于耳石器斑部间质和半规管脊部，以及骨迷路中将树突神经传递到眉部和斑部的通道中。在感觉细胞、暗细胞、无细胞结构、耳石膜或杯状结构中均未检测到Cochlin。这些发现支持Cochlin突变引起的嗜酸性物质在内耳沉积可导致前庭神经继发性树突状退行性变的理论。版权所有(C)2005 S.Karger AG，巴塞尔。
The COCH gene mutated in autosomal dominant sensorineural deafness (DFNA9) encodes cochlin, a major constituent of the inner ear extracellular matrix. Cochlin constitutes 70% of the inner ear protein and cochlin isoforms can be classified into three subgroups, p63s, p44s and p40s. Symptoms of some DFNA9 patients are consistent with those of Meniere's disease. Here, we report the expression of cochlin in the vestibular organ of rats using isoform-specific antibodies that recognize all three isoforms. Cochlin is highly expressed in the stromata of the maculae of otolithic organs and cristae of semicircular canals, and in the channels in the bony labyrinth that transmit the dendritic innervation to the cristae and maculae. Cochlin cannot be detected in the sensory cells, dark cells, nor in the acellular structures, otolithic membrane or in the cupula. These findings support the theory that deposition of acidophilic substance in the inner ear caused by mutation of cochlin can induce a secondary retrograde dendritic degeneration of the vestibular nerves. Copyright (C) 2005 S. Karger AG, Basel.