GJB2基因条件敲除小鼠耳蜗扫描电镜观察

山东大学学报(医学版) ›› 2010, Vol. 48 ›› Issue (12): 19-22.

GJB2基因条件敲除小鼠耳蜗扫描电镜观察

张延平1 ，佟明望1，李丽娜1，张晓强1，孙建和2，林曦3

1. 中国人民解放军第309医院耳鼻喉科, 北京 100091; 2. 中国人民解放军总医院耳鼻喉研究所, 北京 100853;
3. 美国Emory大学耳鼻咽喉－头颈外科/细胞生物系, 亚特兰大 30322

收稿日期:2010-06-28 发布日期:2010-12-16
作者简介:张延平（1972- ），女，医学博士，副主任医师，主要从事耳聋防治的研究。
基金资助:
军队十一五课题（06H048），国家自然科学基金面上项目青年基金（30700936），解放军309医院院内基金资助（LCB08MS12、309-09-03）。

The morphologic presentation of cochlear in GJB2 conditional knock out mice under scanning electron microscopy

ZHANG Yan-ping1, TONG Ming-wang1, LI Li-na1, ZHANG Xiao-qiang1, SUN Jian-he2, LIN Xi3

1. Department of Otolaryngology, 309th Hospital of Chinese PLA, Beijing 100091, China;
2. Department of Otolaryngology Head Neck Surgery, General Hospital of Chinese PLA, Beijing 100853, China;
3. Department of Otolaryngology Head Neck Surgery Cell Biology, Emory University, Atlanta, 30322, USA.

Received:2010-06-28 Published:2010-12-16

摘要/Abstract

摘要：

目的进一步探讨Cx26突变导致耳聋的机制。方法采用扫描电镜观察技术观察GJB2基因条件敲除小鼠模型（cCx26ko mice）小鼠出生后耳蜗表面形态的超微结构变化。结果观察发现P8(出生第9天)和P12（出生第13天）小鼠耳蜗Corti器表面形态、毛细胞以及Deiters细胞等支持细胞均呈正常发育的一个演变过程，P21（出生第22天）小鼠耳蜗Corti器表面形态由底圈到中圈可见外毛细胞广泛散在性静纤毛的融合、脱落及外毛细胞缺失，Corti器网状板皱缩，并可见局部Corti器网状板破损，内毛细胞未见明显的病理变化。结论 GJB2基因缺失可以引起小鼠耳蜗Corti器外毛细胞静纤毛的缺失，Corti器网状板皱缩，局部Corti器网状板破损。对耳蜗结构的影响发生在小鼠出生后21d以前。

关键词: 缝隙连接26；突变；毛细胞，听觉；扫描电子显微镜

Abstract:

Objective To explore the mechanism of hereditary hearing loss. Methods Cochlea from cCx26 mice on P8（the 8th postnatal day）, P12 （the 12th postnatal day）and P21（the 21st postnatal day） were examined with scanning microscope (SEM). Results On P8 and P12, the external morphology of inner hair cells (IHC), outer hair cells (OHC) and supporting cells developed normally. But in P21 stage, extensive coalesce or absence of stereo cilia of OHC and loss of OHC in the basal and middle turn of cochlea were seen. And the cuticular plate was shriveled and damaged， however， the IHCs in the cochlea were intact without any obvious change. Conclusion Mutation of GJB2 may cause the absence of stereo cilia of OHC, the shrinkage and the damage of Corti′s cuticular plate in mice. All these morphologic changes of cochlea may occur before P21.

Key words: Connexin26; Mutation; Hair Cell, Auditory; Scanning Electron Microscopy

中图分类号:

R764.35

张延平1 ，佟明望1，李丽娜1，张晓强1，孙建和2，林曦3. GJB2基因条件敲除小鼠耳蜗扫描电镜观察[J]. 山东大学学报(医学版), 2010, 48(12): 19-22.

ZHANG Yan-ping1, TONG Ming-wang1, LI Li-na1, ZHANG Xiao-qiang1, SUN Jian-he2, LIN Xi3. The morphologic presentation of cochlear in GJB2 conditional knock out mice under scanning electron microscopy[J]. JOURNAL OF SHANDONG UNIVERSITY (HEALTH SCIENCES), 2010, 48(12): 19-22.

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