GJB2基因条件敲除小鼠耳蜗扫描电镜观察  

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

作　　者：张延平[1] 佟明望[1] 李丽娜[1] 张晓强[1] 孙建和[2] 林曦 

机构地区：[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

出　　处：《山东大学学报（医学版）》2010年第12期19-22,共4页Journal of Shandong University:Health Sciences

基　　金：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)

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。结果观察发现出生第9天(P8)和出生第13天(P12)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,出生第22天(P21)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。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 a 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 coalescence or absence of stereo cilia of OHC and loss of OHC in the basal and middle turn of cochlea were seen.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 morphological changes of cochlea may occur before P21.

关 键 词：缝隙连接蛋白26 突变 毛细胞 听觉 扫描电子显微镜 

分 类 号：R764.35[医药卫生—耳鼻咽喉科]