Modeling FXS: Human Pluripotent Stem Cells and In Vitro Neural Differentiation.
Methods Mol Biol
; 1942: 89-100, 2019.
Article
em En
| MEDLINE
| ID: mdl-30900178
ABSTRACT
In fragile X syndrome (FXS) embryos FMRP is widely expressed during early stages of embryogenesis however it is inactivated by the end of the first trimester. In the same manner, human embryonic stem cell (hESC) lines from FXS blastocysts, bearing the full CGG expansion mutation, express FMRP in their pluripotent stage and in neurons derived following in vitro differentiation, FMR1 is completely silenced. Therefore, in vitro neural differentiation of FX-hESC lines serves as a uniquely valuable model system to study the developmental mechanisms underlying FXS, together with the proper differentiation protocol to mimic the neurodevelopmental process occurs in vivo.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Diferenciação Celular
/
Células-Tronco Embrionárias
/
Células-Tronco Pluripotentes Induzidas
/
Síndrome do Cromossomo X Frágil
/
Modelos Biológicos
/
Neurônios
Limite:
Humans
Idioma:
En
Revista:
Methods Mol Biol
Assunto da revista:
BIOLOGIA MOLECULAR
Ano de publicação:
2019
Tipo de documento:
Article
País de afiliação:
Israel