Modeling FXS: Human Pluripotent Stem Cells and In Vitro Neural Differentiation.

Modeling FXS: Human Pluripotent Stem Cells and In Vitro Neural Differentiation. Methods Mol Biol. 2019;1942:89-100 Authors: Kuznitsov-Yanovsky L, Mayshar Y, Ben-Yosef D 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. PMID: 30900178 [PubMed - indexed for MEDLINE]
Source: Mol Biol Cell - Category: Molecular Biology Authors: Tags: Methods Mol Biol Source Type: research