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


Autoria(s): ALVAREZ-SAAVEDRA, Matias; CARRASCO, Loreto; SURA-TRUEBA, Sylvia; AIELLO, Vera Demarchi; WALZ, Katherina; XAVIER NETO, Jose; YOUNG, Juan I.
Contribuinte(s)

UNIVERSIDADE DE SÃO PAULO

Data(s)

19/10/2012

19/10/2012

2010

Resumo

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.

FONDECYT[1061067]

FONDECYT[1051079]

Chilean Government

Antofagasta Minerals

Arauco

Empresas CMPC

Indura

Naviera Ultragas

Telefonica del Sur

Gobierno Regional de Los Rios

Identificador

HUMAN MOLECULAR GENETICS, v.19, n.11, p.2177-2190, 2010

0964-6906

http://producao.usp.br/handle/BDPI/21150

10.1093/hmg/ddq096

http://dx.doi.org/10.1093/hmg/ddq096

Idioma(s)

eng

Publicador

OXFORD UNIV PRESS

Relação

Human Molecular Genetics

Direitos

restrictedAccess

Copyright OXFORD UNIV PRESS

Palavras-Chave #CPG-BINDING PROTEIN #GREEN FLUORESCENT PROTEIN #HOLT-ORAM-SYNDROME #SEVERE MENTAL-RETARDATION #RETT-SYNDROME #CLEIDOCRANIAL DYSPLASIA #DNA METHYLATION #GENE-EXPRESSION #BONE-FORMATION #ELECTROCARDIOGRAPHIC FINDINGS #Biochemistry & Molecular Biology #Genetics & Heredity
Tipo

article

original article

publishedVersion