The fragile X mental retardation protein, FMRP, recognizes G-quartets

Ment Retard Dev Disabil Res Rev. 2004;10(1):49-52. doi: 10.1002/mrdd.20008.

Abstract

Fragile X mental retardation is a disease caused by the loss of function of a single RNA-binding protein, FMRP. Identifying the RNA targets recognized by FMRP is likely to reveal much about its functions in controlling some aspects of memory and behavior. Recent evidence suggests that one of the predominant RNA motifs recognized by the FMRP protein is an intramolecular G-quartet and that the RGG box of FMRP mediates this interaction. Searching databases of mRNA sequence information, as well as compiled sequences of predicted FMRP targets based on biochemical identification, has revealed that many of these predicted FMRP targets contain intramolecular G-quartets. Interestingly, many of the G-quartet containing RNA targets encode proteins involved in neuronal development and synaptic function. Defects in the metabolism of this set of RNAs, presumably in the translation of their protein products, is likely to underlie the behavioral and cognitive changes seen in the disease.

Publication types

  • Review

MeSH terms

  • Animals
  • Base Sequence
  • Female
  • Fragile X Mental Retardation Protein
  • Fragile X Syndrome / genetics*
  • Humans
  • Male
  • Mice
  • Nerve Tissue Proteins / deficiency
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / physiology*
  • Neurons / physiology
  • Protein Biosynthesis*
  • RNA, Messenger / analysis
  • RNA, Messenger / chemistry
  • RNA, Messenger / physiology*
  • RNA-Binding Proteins / chemistry
  • RNA-Binding Proteins / genetics
  • RNA-Binding Proteins / physiology*
  • Rats

Substances

  • FMR1 protein, human
  • Fmr1 protein, mouse
  • Fmr1 protein, rat
  • Nerve Tissue Proteins
  • RNA, Messenger
  • RNA-Binding Proteins
  • Fragile X Mental Retardation Protein