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Por favor, use este identificador para citar o enlazar a este item: http://hdl.handle.net/10261/124191
Título

An increase in MECP2 dosage impairs neural tube formation

AutorPetazzi, Paolo; Akizu, Naiara; García, Alejandra; Estarás, Conchi ; Martínez de Paz, Alexia; Rodríguez-Paredes, Manuel ; Martínez-Balbás, Marian ; Huertas, Dori; Esteller, Manel
Palabras claveRett syndrome
MECP2
Chicken neurogenesis
Neuroblast proliferation
Fecha de publicaciónjul-2014
EditorAcademic Press
CitaciónNeurobiology of Disease 67: 49-56 (2014)
ResumenEpigenetic mechanisms are fundamental for shaping the activity of the central nervous system (CNS). Methyl-CpG binding protein 2 (MECP2) acts as a bridge between methylated DNA and transcriptional effectors responsible for differentiation programs in neurons. The importance of MECP2 dosage in CNS is evident in Rett Syndrome and MECP2 duplication syndrome, which are neurodevelopmental diseases caused by loss-of-function mutations or duplication of the MECP2 gene, respectively. Although many studies have been performed on Rett syndrome models, little is known about the effects of an increase in MECP2 dosage. Herein, we demonstrate that MECP2 overexpression affects neural tube formation, leading to a decrease in neuroblast proliferation in the neural tube ventricular zone. Furthermore, an increase in MECP2 dose provokes premature differentiation of neural precursors accompanied by greater cell death, resulting in a loss of neuronal populations. Overall, our data indicate that correct MECP2 expression levels are required for proper nervous system development. © 2014 .
Versión del editorhttp://dx.doi.org/10.1016/j.nbd.2014.03.009
URIhttp://hdl.handle.net/10261/124191
DOI10.1016/j.nbd.2014.03.009
Identificadoresdoi: 10.1016/j.nbd.2014.03.009
issn: 1095-953X
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