New insights into fragile X syndrome: from molecules to neurobehaviors.

Peng Jin, Stephen T Warren
Author Information
  1. Peng Jin: Department of Human Genetics, Emory University School of Medicine, Atlanta, GA 30322, USA.

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.

Grants

  1. P01 HD35576/NICHD NIH HHS
  2. R37HD20521/NICHD NIH HHS

MeSH Term

Animals
Brain
Cell Nucleus
Fragile X Mental Retardation Protein
Fragile X Syndrome
Humans
Nerve Tissue Proteins
Neuronal Plasticity
Neurons
Nucleic Acid Conformation
Protein Biosynthesis
RNA, Messenger
RNA-Binding Proteins

Chemicals

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

Word Cloud

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