Modeling FXS with mouse neural progenitors

Abstract

The neurosphere assay is a widely used method to culture neural precursor cells (NPCs), which include mixed populations of neural stem and progenitor cells, from the mammalian central nervous system. Fmr1-knockout (KO) mice generated to model fragile X syndrome (FXS) recapitulate the major phenotype of FXS. Neurosphere differentiation of cortical progenitors derived from brains of Fmr1-KO mice has been shown to reflect disordered mechanisms during cortical development in FXS in vivo. The cellular composition of neurospheres is heterogeneous, but robust FXS-specific alterations can be identified when culturing conditions are kept constant.