Cited by 74RNA Research and SplicingRNA and protein synthesis mechanismsDNA and Nucleic Acid Chemistry

The role of G-quadruplex in RNA metabolism: involvement of FMRP and FMR2P

Melko, Mireille, Bardoni, Barbara·2010·Biochimie

Abstract

Regulation of post-transcriptional gene expression is a cellular process that is accomplished through the activity of multiple mRNP (messenger RiboNucleoProtein) complexes which are composed of mRNA-binding proteins and RNA molecules interacting with those proteins. The specificity of these interactions is mediated by the ability of the RNA-binding proteins to precisely recognize and bind RNA sequences or structures. Alterations of their function may have some dramatic consequences, resulting in different pathologies. An increasing body of data is emerging showing the impact of a G-quadruplex forming structure in the maturation and expression of some RNA molecules. We review here the role of the G-quadruplex RNA structure in the regulation of translation and splicing, when it interacts with two RNA-binding proteins: FMRP (Fragile X Mental Retardation Protein) and FMR2P (Fragile X Mental Retardation 2 protein). Impaired expression of these proteins causes two forms of intellectual disability: the Fragile X Mental Retardation syndrome (FXS) and the FRAXE-associated mental retardation (FRAXE), respectively. FMRP is involved in different steps of RNA metabolism and, in particular, in translational regulation. FMR2P has been initially described as a transcription factor and we recently showed also its role in regulation of alternative splicing. By the study of the functional significance of the interaction of both FMRP and FMR2P with a G-quadruplex forming RNA we were able to show…

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Crossref · 10.1016/j.biochi.2010.05.018

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Authors
Melko, M., & Bardoni, B.
Affiliations
Centre National de la Recherche Scientifique (FR)
Corresponding author
Bardoni, Barbara
Venue
Biochimie
Publisher
Elsevier BV
Published
2010-08
Volume
92
Issue
8
Pages
919-926
ISSN
  • 0037-9042 (ISSN-L)
  • 0300-9084
OpenAlex
W1998846172
Cited by
74
References
5
Citations by year
2026: 1 · 2025: 1 · 2023: 5 · 2022: 2 · 2021: 3 · 2020: 5 · 2019: 5 · 2018: 3 · 2017: 4 · 2016: 4 · 2015: 6 · 2014: 8 · 2013: 7 · 2012: 9
FWCI
0.76
Type
journal article
Language
en
Open access
closed
Locations
  • Biochimie (publishedVersion)
  • PubMed (publishedVersion)
  • HAL (submittedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA Research and Splicing
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA and protein synthesis mechanisms
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA and Nucleic Acid Chemistry
Keywords
RNA G-quadruplex, FMRP, RNA-binding proteins, post-transcriptional regulation, translational regulation, alternative splicing, exonic splicing enhancers, fragile X syndrome, FRAXE, intellectual disability, RNA metabolism
MeSH terms
Amino Acid Sequence, Humans, Molecular Sequence Data, RNA, 5' Untranslated Regions, Fragile X Messenger Ribonucleoprotein 1, Fragile X Mental Retardation Protein, G-Quadruplexes

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