Connexin43基因缺陷新生小鼠心脏组织病理学研究  

Histopathological analysis of neonatal mouse hearts with connexin43 gene defects

作　　者：谢利剑[1] 黄国英[1] 赵晓晴[1] 沈媛[1] 周国民[2] 

机构地区：[1]上海复旦大学附属儿科医院心血管中心,200032 [2]上海复旦大学医学院解剖组胚学系

出　　处：《中华医学杂志》2005年第18期1249-1251,共3页National Medical Journal of China

基　　金：教育部博士点基金资助项目(20010246018);上海市曙光计划资助项目(2000SG09)

摘　　要：目的观察不同程度Connexin43基因缺陷对新生小鼠心脏发育的影响。方法采用Cx43基因敲除(Cx43KO)和CMV43CT两种Cx43基因缺陷的小鼠模型,聚合酶链反应鉴定基因型。然后将新生小鼠心脏分离固定,HE染色观察心脏的形态结构。普通C57BL6/SJ小鼠作为对照。结果所有11只纯合型Cx43KO小鼠生后1d内均死亡,心脏表现为严重右室流出道梗阻。在20只纯合型CMV43CT新生小鼠中,有12只生后2d内死亡,其心脏不仅表现有右室流出道梗阻,还出现了房间隔缺损、室间隔缺损等其他心脏畸形;而另外8只未见心脏异常。所有杂合型Cx43KO和CMV43CT基因缺陷的小鼠生后均存活,心脏病理学检测未见异常。结论Cx43基因缺陷与心脏发育异常有密切的关系,但不同类型和不同程度的Cx43基因功能缺失对心脏发育的影响也不尽相同。Objective To investigate the characteristics of heart morphology in neonatal mice with connexin43 gene defects.Methods Two mouse lines were used in this study, which included connexin43 knockout (Cx43 KO) mice and CMV43~CT transgenic mice. PCR analysis was carried out to identify the genotypes of two transgenic mice. Sections with HE staining were analyzed to display the morphologic structures of the hearts in neonatal mice. C57BL6/SJ mice were used as control.Results All 11 homozygous Cx43 KO mice died within 24 hr after birth showing severe right ventricular outflow tract obstruction (RVOTO). Out of 20 homozygous CMV43~CT transgenic mice, 12 mice died within 48 hr after birth showing not only RVOTO, but also atrial septal defect (ASD), ventricular septal defect (VSD) and other cardiac defects, while the remaining 8 mice were alive without heart defects. Both heterozygous Cx43 KO and CMV43~CT transgenic mice had normal hearts.Conclusion Connexin43 gene defect is obviously associated with abnormal heart morphogenesis. The different types and different dosage of the gene defects may lead to different phenotypes of hearts.

关 键 词：CONNEXIN43 基因缺陷 新生小鼠 组织病理学研究 流出道梗阻 聚合酶链反应 C57BL6 CX43基因 心脏发育 不同程度 房间隔缺损 室间隔缺损 病理学检测 基因敲除 小鼠模型 形态结构 染色观察 心脏表现 心脏畸形 心脏异常 发育异常 

分 类 号：R541[医药卫生—心血管疾病]