Elevated expression of MeCP2 in cardiac and skeletal tissues is detrimental for normal development

被引：40

作者：

Alvarez-Saavedra, Matias ^{[1
]}

Carrasco, Loreto ^{[1
]}

Sura-Trueba, Sylvia ^{[3
]}

Aiello, Vera Demarchi ^{[2
]}

Walz, Katherina ^{[1
,4
]}

Xavier Neto, Jose ^{[3
]}

Young, Juan I. ^{[1
,4
,5
]}

机构：

[1] Ctr Estudios Cient, Valdivia 5110246, Chile

[2] Univ Sao Paulo, Heart Inst InCor, Pathol Lab, BR-05403000 Sao Paulo, Brazil

[3] Univ Sao Paulo, Heart Inst InCor, Lab Genet & Mol Cardiol, BR-05403000 Sao Paulo, Brazil

[4] Univ Miami, John P Hussman Inst Human Genom, Miller Sch Med, Miami, FL 33136 USA

[5] CIN Ctr Ingn Innovac CECS, Valdivia 5110246, Chile

来源：

HUMAN MOLECULAR GENETICS | 2010年 / 19卷 / 11期

关键词：

CPG-BINDING PROTEIN; GREEN FLUORESCENT PROTEIN; SEVERE MENTAL-RETARDATION; RETT-SYNDROME; DNA METHYLATION; BONE-FORMATION; ELECTROCARDIOGRAPHIC FINDINGS; CLEIDOCRANIAL DYSPLASIA; CYTOSINE METHYLATION; BDNF TRANSCRIPTION;

D O I：

10.1093/hmg/ddq096

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

MeCP2 plays a critical role in interpreting epigenetic signatures that command chromatin conformation and regulation of gene transcription. In spite of MeCP2's ubiquitous expression, its functions have always been considered in the context of brain physiology. In this study, we demonstrate that alterations of the normal pattern of expression of MeCP2 in cardiac and skeletal tissues are detrimental for normal development. Overexpression of MeCP2 in the mouse heart leads to embryonic lethality with cardiac septum hypertrophy and dysregulated expression of MeCP2 in skeletal tissue produces severe malformations. We further show that MeCP2's expression in the heart is developmentally regulated; further suggesting that it plays a key role in regulating transcriptional programs in non-neural tissues.

引用

页码：2177 / 2190

页数：14

共 100 条

[1] Cardiac disease and Rett syndrome [J].