Ultrastructural pathological changes in the cochlear cells of connexin 26 conditional knockout mice.
Ultrastructural pathological changes in the cochlear cells of connexin 26 conditional knockout mice.
复制标题
Connexin 26条件敲除小鼠耳蜗细胞超微结构病理变化
DOI：
10.3892/mmr.2013.1614
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发表时间：
2013-10
影响因子：
3.4
通讯作者：
Li HW
中科院分区：
文献类型：
--
作者：
Lin L;Wang YF;Wang SY;Liu SF;Yu Z;Xi L;Li HW
连接蛋白26 （Cx26）基因突变是人类非综合征遗传性耳聋的最常见原因。由Cx26引起的耳聋发病机制尚不清楚。为了探讨Cx26基因缺失突变的基本机制，本研究观察了Cx26基因条件敲除小鼠耳蜗感觉上皮超微结构的变化和一些标记蛋白。耳蜗标本取自Cx26条件敲除小鼠（cCx26ko），野生型小鼠作为对照。在耳蜗感觉上皮的不同细胞中分别标记柱细胞标记物P75、支持细胞标记物prox1和毛细胞标记物myosin 6和phalloidin抗体。透射电镜观察耳蜗感觉上皮超微结构形态。通过对毛细胞和支持细胞的耳蜗感觉上皮细胞标记物的观察，发现cCx26基因敲除小鼠早期未见明显变化，而Corti和Nuel空间器官的隧道在听力发作前未发育。从出生后第10天开始观察细胞死亡（P10）。在耳蜗中观察到的唯一存活细胞区域是Hensen细胞区，在P180后出现小胶质样细胞。总体而言，本研究显示cCx26ko小鼠耳蜗感觉上皮超微结构形态异常。小胶质样细胞可能参与了cCx26ko小鼠的细胞变性过程。
Mutations in the gene of connexin 26 (Cx26) are the most common cause of human non-syndromic hereditary deafness. The pathogenesis of deafness caused by Cx26 remains uncertain. To explore the basic mechanism underlying Cx26 null mutations, ultrastructural changes and a number of marker proteins in the cochlear sensory epithelium of Cx26 conditional knockout mice were observed in the current study. Cochlear specimens were obtained from Cx26 conditional knockout mice (cCx26ko), while wild-type mice served as controls. Antibodies against the pillar cell marker P75, the supporting cell marker prox1 and hair cell markers myosin 6 and phalloidin were labeled in different cells of the cochlear sensory epithelium of cochlear cryosections. The ultrastructural morphology of cochlear sensory epithelium was observed using transmission electron microscopy. Following the observation of cochlear sensory epithelium cell markers for hair cells and supporting cells, no significant changes were observed at the early stage, while the tunnel of the organ of Corti and Nuel’s space was not developed prior to hearing onset in cCx26 knockout mice. Cell death was observed from postnatal day 10 (P10). The only region of surviving cells observed in the cochlea was the Hensen cell region, where microglia-like cells appeared following P180. Overall, the present study showed an abnormal ultrastructural morphology in the cochlear sensory epithelium in cCx26ko mice. Microglia-like cells may be involved in the process of cell degeneration in cCx26ko mice.
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DOI：
10.1016/j.bbrc.2009.05.023
发表时间：
2009-07-17
影响因子：
3.1
作者：
Wang, Yunfeng;Chang, Qing;Tang, Wenxue;Sun, Yu;Zhou, Binfei;Li, Huawei;Lin, Xi
通讯作者：
Lin, Xi
影响因子：
1.5
作者：
Ohyama, T;Groves, AK
通讯作者：
Groves, AK
影响因子：
7.8
作者：
Gabriel, HD;Jung, D;Bützler, C;Temme, A;Traub, O;Winterhager, E;Willecke, K
通讯作者：
Willecke, K
影响因子：
3.3
作者：
Inoshita, A.;Iizuka, T.;Ikeda, K.
通讯作者：
Ikeda, K.
DOI：
10.1073/pnas.0501859102
发表时间：
2005-10-18
影响因子：
11.1
作者：
Zhang, YP;Tang, WX;Lin, X
通讯作者：
Lin, X