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Current Genomics

Editor-in-Chief

ISSN (Print): 1389-2029
ISSN (Online): 1875-5488

Fragile X Mental Retardation Protein: Many Partners and Multiple Targets for a Promiscuous Function

Author(s): E. W. Khandjian, E. Bechara, L. Davidovic and B. Bardoni

Volume 6, Issue 7, 2005

Page: [515 - 522] Pages: 8

DOI: 10.2174/138920205775067701

Price: $65

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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


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