GRP78/Bip在GJB2基因条件敲除小鼠耳蜗中的表达

The Expression of GRP78/Bip in GJB2 Conditional Knock out Mice Cochlea

张延平;张晓强;刘雅丽;黄玮;孙玉蕊;

1:中国人民解放军第309医院耳鼻喉科

2:河南省医学情报研究所

3:河南省卫生厅

摘要
目的研究GRP78/Bip在GJB2基因条件敲除小鼠耳蜗中的表达,探讨内质网应激(endoplasmic reticulum stress,ERS)与非综合征型聋的相关性。方法敲基因小鼠(6只)Cx26loxp/loxp;Pax2 Cre/+(cCx26)为实验组,野生型BALB/c小鼠(6只)为正常对照组,两组分别选取P8、P12和P21小鼠各2只进行实验。采用耳蜗冰冻切片,免疫组化法检测耳蜗切片中葡萄糖调节蛋白78(glucose regulated protein,GRP78)的表达。结果 cCx26小鼠耳蜗的GRP78/Bip表达定位与对照组相同,但在P8和P12时cCx26小鼠耳蜗表达更强,到P21时,在盖膜、耳蜗外侧壁GRP78/Bip表达较对照组减弱,而在顶回仅存的不成熟Corti细胞团中,GRP78/Bip表达与对照组接近。结论 GJB2基因条件敲除小鼠耳蜗发育成熟之前,其耳蜗中GRP78/Bip表达增高,ERS可能参与了GJB2基因突变导致的非综合征型聋的发生过程。
关键词
缝隙连接蛋白26;小鼠;基因敲除;内质网;分子伴侣
基金项目(Foundation):
全军十一五项目归国人员课题(06H048);; 国家自然科学基金面上项目青年基金(30700936);; 中国人民解放军第309医院院内基金(LCB08MS12、309-09-03)联合资助
作者
张延平;张晓强;刘雅丽;黄玮;孙玉蕊;
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