MECP2
胶质纤维酸性蛋白
星形胶质细胞
雷特综合征
生物
扁桃形结构
内科学
内分泌学
下丘脑
波形蛋白
神经科学
中枢神经系统
医学
表型
遗传学
免疫学
基因
免疫组织化学
作者
Robin M. Forbes-Lorman,Joseph R. Kurian,Anthony P. Auger
出处
期刊:Brain Research
[Elsevier]
日期:2014-01-01
卷期号:1543: 151-158
被引量:32
标识
DOI:10.1016/j.brainres.2013.11.011
摘要
Mutations in MECP2 cause Rett syndrome (RTT), an X-linked neurodevelopmental disorder that primarily affects females. Individuals with RTT have increased glial fibrillary acidic protein (GFAP) expression in the brain. GFAP is an intermediate filament protein that is expressed predominately within astrocytes in the CNS. MeCP2 binds to methylated regions of the GFAP promoter region and suppresses GFAP expression in vitro. Therefore, we wanted to determine if transiently reducing MeCP2 expression would increase GFAP expression in the developing rat brain. Male and female rats received infusions of either MeCP2 or control siRNA targeting the amygdala during the first 3 days of postnatal life. Brains were collected after 6 h or 2 weeks following the last infusion. MeCP2 siRNA increased GFAP mRNA and protein within the female, but not the male, amygdala on postnatal day (PN) 2. Two weeks following the infusion, levels returned to normal. MeCP2 siRNA targeting the hypothalamus also increases GFAP mRNA within the female hypothalamus on PN2, suggesting that the regulation is not brain region-specific. It appears that MeCP2 does not regulate all astrocyte markers in the developing female brain, but specifically regulates GFAP expression, as levels of S100β and vimentin were not altered in the female amygdala at either time point. These data contribute to the idea that the role of MeCP2 differs in the developing male versus female brain. Further elucidating the regulation and function of GFAP can contribute to our understanding of MeCP2 function and perhaps RTT etiology.
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