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Type: Journal article
Title: FRAXE-associated mental retardation protein (FMR2) is an RNA-binding protein with high affinity for G-quartet RNA forming structure
Author: Bensaid, M.
Melko, M.
Bechara, E.
Davidovic, L.
Berretta, A.
Catania, M.
Gecz, J.
Lalli, E.
Bardoni, B.
Citation: Nucleic Acids Research, 2009; 37(4):1269-1279
Publisher: Oxford Univ Press
Issue Date: 2009
ISSN: 0305-1048
Statement of
Mounia Bensaid, Mireille Melko, Elias G. Bechara, Laetitia Davidovic, Antonio Berretta, Maria Vincenza Catania, Jozef Gecz, Enzo Lalli, and Barbara Bardoni
Abstract: FRAXE is a form of mild to moderate mental retardation due to the silencing of the FMR2 gene. The cellular function of FMR2 protein is presently unknown. By analogy with its homologue AF4, FMR2 was supposed to have a role in transcriptional regulation, but robust evidences supporting this hypothesis are lacking. We observed that FMR2 co-localizes with the splicing factor SC35 in nuclear speckles, the nuclear regions where splicing factors are concentrated, assembled and modified. Similarly to what was reported for splicing factors, blocking splicing or transcription leads to the accumulation of FMR2 in enlarged, rounded speckles. FMR2 is also localized in the nucleolus when splicing is blocked. We show here that FMR2 is able to specifically bind the G-quartet-forming RNA structure with high affinity. Remarkably, in vivo, in the presence of FMR2, the ESE action of the G-quartet situated in mRNA of an alternatively spliced exon of a minigene or of the putative target FMR1 appears reduced. Interestingly, FMR1 is silenced in the fragile X syndrome, another form of mental retardation. All together, our findings strongly suggest that FMR2 is an RNA-binding protein, which might be involved in alternative splicing regulation through an interaction with G-quartet RNA structure.
Keywords: Cells, Cultured
Cell Line
Cell Nucleus Structures
RNA-Binding Proteins
Nuclear Proteins
Alternative Splicing
Protein Structure, Tertiary
Fragile X Mental Retardation Protein
DOI: 10.1093/nar/gkn1058
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Paediatrics publications

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