Journal
MENTAL RETARDATION AND DEVELOPMENTAL DISABILITIES RESEARCH REVIEWS
Volume 10, Issue 1, Pages 49-52Publisher
WILEY-LISS
DOI: 10.1002/mrdd.20008
Keywords
fragile X mental retardation protein (FMRP); RNA binding protein; intramolecular G-quartet; in vitro RNA selection; translational control
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Funding
- NICHD NIH HHS [R01 HD040647] Funding Source: Medline
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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. (C) 2004 Wiley-Liss, Inc.
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