Downregulation of CNPase in a MeCP2 deficient mouse model of Rett syndrome
Downregulation of CNPase in a MeCP2 deficient mouse model of Rett syndrome
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DOI：
10.1179/016164111x13214359296301
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发表时间：
2012-01
影响因子：
1.9
通讯作者：
Wei Wu;W. Gu;X. Xu;S. Shang;Zhengyan Zhao
中科院分区：
文献类型：
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作者：
Wei Wu;W. Gu;X. Xu;S. Shang;Zhengyan Zhao
摘要目的：探讨甲基CpG结合蛋白2(MeCP2)可能与Rett综合征(RTT)相关的靶基因。方法：取Mecp2308/Y小鼠和对照组小鼠的脑组织，进行实时定量逆转录聚合酶链式反应(RT-PCR)、免疫组织化学染色和Western印迹分析连接蛋白(Cx)43、Cx45、Cx40、Cx32、2，3-环核苷酸3-磷酸水解酶(CNPase)和胶质纤维酸性蛋白(GFAP)。结果：RTT组小鼠皮质下白质和海马区CNPase的表达在mRNA和蛋白水平均低于对照组。相反，与对照组相比，RTT小鼠Cx43、Cx40、Cx45、Cx32或GFAP的表达没有改变。结论：脑内CNPase表达下调可能是MECP2基因突变的结果，皮质下白质和海马区少突胶质细胞功能障碍可能参与了RTT的发病机制。
Abstract Objectives: To investigate the possible target genes of methyl-CpG-binding protein 2 (MeCP2) that contribute to Rett syndrome (RTT). Methods: Brain tissues were taken from Mecp2308/Y mice or control mice and then subjected to real-time quantitative reverse transcriptase polymerase chain reaction (RT-PCR), immunohistochemical staining, and Western blot analysis for connexin (Cx)43, Cx45, Cx40, Cx32, 2,3-cyclic nucleotide 3-phosphohydrolase (CNPase), and glial fibrillary acidic protein (GFAP). Results: The expression of CNPase in subcortical white matter and hippocampi was lower in RTT mice compared to control mice at both mRNA and protein levels. In contrast, the expression of Cx43, Cx40, Cx45, Cx32, or GFAP was not altered in RTT mice compared to control mice. Conclusion: The downregulation of CNPase expression in the brain may be a possible consequence of MECP2 gene mutation, and the indicated dysfunction of the oligodendrocytes in the subcortical white matter and hippocampi may be involved in RTT pathogenesis.