Modeling FXS with Mouse Neural Progenitors

Methods Mol Biol. 2019:1942:71-78. doi: 10.1007/978-1-4939-9080-1_6.

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.

Keywords: Brain; Cell culture; Differentiation; Mouse; Neural progenitor; Neurosphere.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Brain / cytology
  • Brain / metabolism*
  • Cell Differentiation*
  • Cells, Cultured
  • Disease Models, Animal*
  • Fragile X Mental Retardation Protein / genetics
  • Fragile X Mental Retardation Protein / metabolism*
  • Fragile X Syndrome / genetics*
  • Humans
  • Mice
  • Mice, Knockout
  • Neural Stem Cells / cytology
  • Neural Stem Cells / metabolism*
  • Neurogenesis*

Substances

  • Fmr1 protein, mouse
  • Fragile X Mental Retardation Protein