New insights into fragile X syndrome: from molecules to neurobehaviors

Trends Biochem Sci. 2003 Mar;28(3):152-8. doi: 10.1016/S0968-0004(03)00033-1.

Abstract

Fragile X syndrome - a common form of inherited mental retardation - is caused by the loss of the fragile X mental retardation 1 protein (FMRP). FMRP is an RNA-binding protein which forms a messenger ribonucleoprotein (mRNP) complex that associates with translating polyribosomes. It has been proposed that FMRP is involved in synaptic plasticity through the regulation of mRNA transportation and translation. Recent advances in the identification of the mRNA ligands that are bound by FMRP, the RNA sequence and structure required for FMRP-RNA interaction, and the physiological consequences of FMRP deficiency in the brain are important steps towards understanding the molecular pathogenesis of fragile X syndrome, and learning and memory in general.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Animals
  • Brain / physiopathology
  • Cell Nucleus / metabolism
  • Fragile X Mental Retardation Protein
  • Fragile X Syndrome* / genetics
  • Fragile X Syndrome* / metabolism
  • Fragile X Syndrome* / pathology
  • Fragile X Syndrome* / physiopathology
  • Humans
  • Nerve Tissue Proteins / chemistry
  • Nerve Tissue Proteins / genetics
  • Nerve Tissue Proteins / metabolism*
  • Neuronal Plasticity
  • Neurons / metabolism*
  • Nucleic Acid Conformation
  • Protein Biosynthesis
  • RNA, Messenger / chemistry
  • RNA, Messenger / metabolism
  • RNA-Binding Proteins*

Substances

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