Sox10 expressing cells in the lateral wall of the aged mouse and human cochlea.

Sox10 expressing cells in the lateral wall of the aged mouse and human cochlea.
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老年小鼠和人耳蜗侧壁中表达 Sox10 的细胞

DOI:
10.1371/journal.pone.0097389
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发表时间:
2014
期刊:
影响因子:
3.7
通讯作者:
Lang H
Lang H
中科院分区:
综合性期刊3区
文献类型:
--
作者:
Hao X;Xing Y;Moore MW;Zhang J;Han D;Schulte BA;Dubno JR;Lang H

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老年性耳聋是一种常见的人类疾病,每三个60岁及以上的美国人中就有一个受到影响。以往的研究表明,老年性聋与耳蜗外侧壁非感觉细胞的丧失有关。Sox 10是一种转录因子,对耳蜗中神经嵴衍生细胞(包括一些非感觉细胞类型)的发育和维持至关重要。已知Sox 10基因的突变会导致人类听力损失和色素沉着缺陷的各种组合。本研究探讨了Sox 10基因表达与老年CBA/CaJ小鼠耳蜗外侧壁和老年人颞骨病理变化之间的潜在关系。用免疫组化定量分析和透射电镜检查了从年轻成年(1-3月龄)和老年(2-2.5岁)小鼠以及人颞骨供体制备的骨组织。在小鼠和人耳蜗的血管纹、外沟和螺旋突起中均存在表达Sox 10的细胞。Sox 10+细胞类型包括边缘细胞、中间细胞和外沟细胞,包括与中阶交界的细胞和延伸到螺旋韧带中的根突(根细胞)的细胞。免疫染色定量分析显示,Sox 10+边缘细胞和外沟细胞在老年小鼠的数量显着减少。电子显微镜评价显示老年小鼠中存活的Sox 10+细胞发生退行性改变。来自87岁及以上供体的人耳蜗中的Strial边缘细胞仅显示Sox 10的弱免疫染色。Sox 10表达水平的下降和Sox 10+细胞在小鼠和人的老年耳朵的损失表明Sox 10在维持侧壁的结构和功能完整性的重要作用。Sox 10+细胞的损失也可能与老年人耳中非感觉细胞修复能力的下降有关。
Age-related hearing loss (presbycusis) is a common human disorder, affecting one in three Americans aged 60 and over. Previous studies have shown that presbyacusis is associated with a loss of non-sensory cells in the cochlear lateral wall. Sox10 is a transcription factor crucial to the development and maintenance of neural crest-derived cells including some non-sensory cell types in the cochlea. Mutations of the Sox10 gene are known to cause various combinations of hearing loss and pigmentation defects in humans. This study investigated the potential relationship between Sox10 gene expression and pathological changes in the cochlear lateral wall of aged CBA/CaJ mice and human temporal bones from older donors. Cochlear tissues prepared from young adult (1–3 month-old) and aged (2–2.5 year-old) mice, and human temporal bone donors were examined using quantitative immunohistochemical analysis and transmission electron microscopy. Cells expressing Sox10 were present in the stria vascularis, outer sulcus and spiral prominence in mouse and human cochleas. The Sox10+ cell types included marginal and intermediate cells and outer sulcus cells, including those that border the scala media and those extending into root processes (root cells) in the spiral ligament. Quantitative analysis of immunostaining revealed a significant decrease in the number of Sox10+ marginal cells and outer sulcus cells in aged mice. Electron microscopic evaluation revealed degenerative alterations in the surviving Sox10+ cells in aged mice. Strial marginal cells in human cochleas from donors aged 87 and older showed only weak immunostaining for Sox10. Decreases in Sox10 expression levels and a loss of Sox10+ cells in both mouse and human aged ears suggests an important role of Sox10 in the maintenance of structural and functional integrity of the lateral wall. A loss of Sox10+ cells may also be associated with a decline in the repair capabilities of non-sensory cells in the aged ear.
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发表时间: 1966-01-01
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DOI: 10.1371/journal.pone.0017949
发表时间: 2011-03-14
期刊: PloS one
影响因子: 3.7
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