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

ALVAREZ-SAAVEDRA, MATÍAS; CARRASCO, LORETO; SURA-TRUEBA, SYLVIA; AIELLO, VERA DEMARCHI; WALZ, KATHERINA; NETO, JOSÉ XAVIER; YOUNG, JUAN I.

HUMAN MOLECULAR GENETICS

OXFORD UNIV PRESS

Año: 2010 vol. 19 p. 2177 - 2190

0964-6906

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. © The Author 2010. Published by Oxford University Press.