Abstract
Fragile X syndrome is the most common inherited form of mental retardation and is due to the silencing of FMR1 gene coding for the FMRP protein. FMRP is an RNA binding protein endowed with Nuclear Localization and Nuclear Export Signals and is associated with actively translating polysomes as part of mRNP complexes. During the past years, efforts from many laboratories to unravel the function of this protein, resulted in the identification of several proteins (mostly RNA-binding) and few hundred of mRNAs that are targeted by FMRP. The puzzle illustrating the FMRP role depicts a protein implicated in different steps of mRNA metabolism. However, its precise mechanism of action is not still understood and the specificity of its function is probably dependent on RNA and/or proteins that interact and associate with it.
Keywords: Fragile X syndrome, mental retardation, RNA-binding domains, RNA-binding proteins, mRNP-complexes, G-quartet, kissing-complex, Rac pathway
Current Genomics
Title: Fragile X Mental Retardation Protein: Many Partners and Multiple Targets for a Promiscuous Function
Volume: 6 Issue: 7
Author(s): E. W. Khandjian, E. Bechara, L. Davidovic and B. Bardoni
Affiliation:
Keywords: Fragile X syndrome, mental retardation, RNA-binding domains, RNA-binding proteins, mRNP-complexes, G-quartet, kissing-complex, Rac pathway
Abstract: Fragile X syndrome is the most common inherited form of mental retardation and is due to the silencing of FMR1 gene coding for the FMRP protein. FMRP is an RNA binding protein endowed with Nuclear Localization and Nuclear Export Signals and is associated with actively translating polysomes as part of mRNP complexes. During the past years, efforts from many laboratories to unravel the function of this protein, resulted in the identification of several proteins (mostly RNA-binding) and few hundred of mRNAs that are targeted by FMRP. The puzzle illustrating the FMRP role depicts a protein implicated in different steps of mRNA metabolism. However, its precise mechanism of action is not still understood and the specificity of its function is probably dependent on RNA and/or proteins that interact and associate with it.
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Cite this article as:
Khandjian W. E., Bechara E., Davidovic L. and Bardoni B., Fragile X Mental Retardation Protein: Many Partners and Multiple Targets for a Promiscuous Function, Current Genomics 2005; 6 (7) . https://dx.doi.org/10.2174/138920205775067701
DOI https://dx.doi.org/10.2174/138920205775067701 |
Print ISSN 1389-2029 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5488 |
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