Heads-up: new roles for the fragile X mental retardation protein in neural stem and progenitor cells

Genesis. 2011 Jun;49(6):424-40. doi: 10.1002/dvg.20745. Epub 2011 May 31.

Abstract

Fragile X syndrome (FXS) is the most common form of inherited mental retardation and is caused by the loss of function for Fragile X Mental Retardation Protein (FMRP), a selective RNA-binding protein with a demonstrated role in the localized translation of target mRNAs at synapses. Several recent studies provide compelling evidence for a new role of FMRP in the development of the nervous system, during neurogenesis. Using a multi-faceted approach and a variety of model systems ranging from cultured neurospheres and progenitor cells to in vivo Drosophila and mouse models these reports indicate that FMRP is required for neural stem and progenitor cell proliferation, differentiation, survival, as well as regulation of gene expression. Here we compare and contrast these recent reports and discuss the implications of FMRP's new role in embryonic and adult neurogenesis, including the development of novel therapeutic approaches to FXS and related neurological disorders such as autism.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Cell Differentiation / physiology*
  • Cell Proliferation
  • Cell Survival / physiology*
  • Drosophila
  • Fragile X Mental Retardation Protein / metabolism
  • Fragile X Mental Retardation Protein / physiology*
  • Gene Expression Regulation / physiology*
  • Humans
  • Mice
  • Neural Stem Cells / metabolism
  • Neural Stem Cells / physiology*
  • Neurogenesis / genetics
  • Neurogenesis / physiology*
  • RNA, Messenger / metabolism*

Substances

  • RNA, Messenger
  • Fragile X Mental Retardation Protein