The role of G-quadruplex in RNA metabolism: involvement of FMRP and FMR2P
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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RNA G-quadruplex-forming structures are involved in several steps of mRNA metabolism.
“RNA G-quadruplex-forming structures are involved in several steps of mRNA metabolism (Melko and Bardoni, 2010; Subramanian et al., 2011).”
From New insights into the regulatory function of CYFIP1 in the context of WAVE- and FMRP-containing complexes (2017) · cites this paper as Melko and Bardoni, 2010
The FMRP-G-quadruplex complex tightly blocks the advance of polyribosomes.
“This probably happens because the FMRP-G-quadruplex complex tightly blocks the advance of polyribosomes (Melko and Bardoni, 2010; for review).”
From New insights into the regulatory function of CYFIP1 in the context of WAVE- and FMRP-containing complexes (2017) · cites this paper as Melko and Bardoni, 2010
The translational repression function of G-quadruplex is reinforced by the presence of FMRP.
“This function is reinforced by the presence of FMRP (Kumari et al., 2007; Melko and Bardoni, 2010).”
From New insights into the regulatory function of CYFIP1 in the context of WAVE- and FMRP-containing complexes (2017) · cites this paper as Melko and Bardoni, 2010
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“This function is reinforced by the presence of FMRP (Kumari et al., 2007; Melko and Bardoni, 2010).”
InfluentialSelf-citation
- Authors
- Melko, M., & Bardoni, B.
- Affiliations
- Centre National de la Recherche Scientifique (FR)
- Corresponding author
- Bardoni, Barbara
- ORCID
- 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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