Objective To study the expression of GRP78/Bip in GJB2 conditional knockout mice (cCx26) cochlea and explore the relationship between nonsyndromic hearing loss and endcplasmic reticulum stress (ERS). Methods Cochlea from P8,P12 and P21 of cCx26(Cx26kxp/kxp; Pax2 Cre/+) and wild type ones (BALB/c) were harvested. The expression of GRP78/Bip was detected by cryosection and immunohistology. Results The expression pattern of GRP78/Bip was almost the same in cCx26 mice and in wild type ones. But the intensity of the expression was higher in the cochlea of cCx26 than that of wild type ones in P8 and P12. In P21, the expression of GRP78/Bip in tectorial membrane and lateral wall of cochlea in cCx26 mice was weaker than that of wild type ones. But in the immature organ of Cortis of apical turn in cCx26 cochlea, the expression was almost the same as that of wild type ones. Conclusion FIRS may play a role in the nonsyndromic hearing loss caused by GJB2 mutation.%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)的表选.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、耳蜗外侧璧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.
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