2024-03-28T22:14:35Zhttp://digital.csic.es/dspace-oai/requestoai:digital.csic.es:10261/1241912019-03-18T15:14:44Zcom_10261_41com_10261_1col_10261_294
Petazzi, Paolo
Akizu, Naiara
García, Alejandra
Estarás, Conchi
Martínez de Paz, Alexia
Rodríguez-Paredes, Manuel
Martínez-Balbás, Marian
Huertas, Dori
Esteller, Manel
2015-10-30T11:14:05Z
2015-10-30T11:14:05Z
2014-07
Neurobiology of Disease 67: 49-56 (2014)
http://hdl.handle.net/10261/124191
10.1016/j.nbd.2014.03.009
http://dx.doi.org/10.13039/501100002809
http://dx.doi.org/10.13039/501100000780
http://dx.doi.org/10.13039/501100006373
http://dx.doi.org/10.13039/100008666
http://dx.doi.org/10.13039/501100000781
http://dx.doi.org/10.13039/100007353
Epigenetic 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 .
eng
openAccess
Rett syndrome
MECP2
Chicken neurogenesis
Neuroblast proliferation
An increase in MECP2 dosage impairs neural tube formation
artículo