Fragile X mental retardation protein targets G quartet mRNAs important for neuronal function

Darnell, Jennifer C.; Jensen, Kirk B.; Jin, Peng; Brown, Victoria; Warren, Stephen T.; Darnell, Robert B.

doi:10.1016/s0092-8674(01)00566-9

Cited by 952Genetics and Neurodevelopmental DisordersRNA Research and SplicingRNA and protein synthesis mechanisms

Fragile X mental retardation protein targets G quartet mRNAs important for neuronal function

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Darnell, Jennifer C., Jensen, Kirk B., Jin, Peng, Brown, Victoria, Warren, Stephen T., Darnell, Robert B.·2001·Cell

DOI 10.1016/s0092-8674(01)00566-9

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Abstract

Loss of fragile X mental retardation protein (FMRP) function causes the fragile X mental retardation syndrome. FMRP harbors three RNA binding domains, associates with polysomes, and is thought to regulate mRNA translation and/or localization, but the RNAs to which it binds are unknown. We have used RNA selection to demonstrate that the FMRP RGG box binds intramolecular G quartets. This data allowed us to identify mRNAs encoding proteins involved in synaptic or developmental neurobiology that harbor FMRP binding elements. The majority of these mRNAs have an altered polysome association in fragile X patient cells. These data demonstrate that G quartets serve as physiologically relevant targets for FMRP and identify mRNAs whose dysregulation may underlie human mental retardation.

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FMRP binds to G-rich sequences (G-quartets) with consensus site DWGG-N(0-2)-DWGG-N(0-1)-DWGG-N(0-1)-DWGG.
“FMRP binds to G-rich sequences (so-called G-quartets; consensus site: DWGG-N(0–2)-DWGG-N(0–1)-DWGG-N(0–1)-DWGG, where D is any nucleotide except C and W is A or U) [17] arranged in a planar conformation and stabilized by Hoogsteen-type hydrogen bonds.”
From FMRP Mediates mGluR5-Dependent Translation of Amyloid Precursor Protein (2007) · cites this paper as [17]
FMRP mostly acts as an mRNA-binding protein that represses translation of a subset of mRNAs.
“Encoded by the FMR1 gene, Fragile X mental retardation protein (FMRP) mostly operates as an mRNA-binding protein (Fig. 5B2) that represses translation of an emerging subset of mRNAs (Brown et al., 2001; Darnell et al., 2001; Darnell et al., 2011; Santoro et al., 2012), among other functions (Contractor et al., 2015).”
From Unifying Views of Autism Spectrum Disorders: A Consideration of Autoregulatory Feedback Loops (2016) · cites this paper as Darnell et al., 2001
FMRP binds RNA in vitro with high affinity for kissing complex and G-quadruplex motifs through its KH and RGG-type RNA-binding domains.
“FMRP binds to RNA in vitro (Siomi et al., 1993) with high affinity for kissing complex and G-quadruplex motifs mediated through its KH and RGG-type RNA binding domains, respectively (Darnell et al., 2005a; Darnell et al., 2001).”
From FMRP Stalls Ribosomal Translocation on mRNAs Linked to Synaptic Function and Autism (2011) · cites this paper as Darnell et al., 2001

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Authors: Darnell, J. C., Jensen, K. B., Jin, P., Brown, V., Warren, S. T., & Darnell, R. B.
Affiliations: Rockefeller University (US)
Howard Hughes Medical Institute (US)
Corresponding author: Darnell, Robert B.
ORCID: Jensen, Kirk B.
Jin, Peng
Warren, Stephen T.
Darnell, Robert B.
Venue: Cell
Publisher: Elsevier BV
Published: 2001-11
Volume: 107
Issue: 4
Pages: 489-499
ISSN: 0092-8674 (ISSN-L)
DOI: 10.1016/s0092-8674(01)00566-9
OpenAlex: W2110811750
Cited by: 952
References: 87
Citations by year: 2026: 12 · 2025: 13 · 2024: 22 · 2023: 21 · 2022: 23 · 2021: 32 · 2020: 36 · 2019: 30 · 2018: 30 · 2017: 38 · 2016: 31 · 2015: 42 · 2014: 38 · 2013: 44 · 2012: 38
FWCI: 30.05
Type: journal article
Language: en
Open access: bronze
doi.org
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
www.elsevier.com/open-access/userlicense/1.0/ (vor, 4276 d)
Locations: Cell (publishedVersion)
PubMed (publishedVersion)
Adelaide Research & Scholarship (AR&S) (University of Adelaide) (submittedVersion)
Funders: National Institute of Neurological Disorders and Stroke (R01NS034389)
Eunice Kennedy Shriver National Institute of Child Health and Human Development (R37HD020521, R01HD040647, P01HD035576)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
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
Keywords: FMRP, fragile X syndrome, G-quartet, RGG box, RNA binding, polysome association, mRNA translation regulation, mRNA localization, synaptic proteins, neuronal development, RNA selection
MeSH terms: Base Sequence, Binding Sites, Codon, Dendrites, Fragile X Syndrome, Genetic Vectors, Humans, Ligands, Molecular Sequence Data, Nerve Tissue Proteins, Neurons, Nucleic Acid Conformation, Protein Binding, Regulatory Sequences, Nucleic Acid, Ribosomes, RNA, Messenger, Synapses, Protein Biosynthesis, Mutagenesis, Consensus Sequence, Sequence Alignment, RNA-Binding Proteins, Protein Structure, Tertiary, Nucleopolyhedroviruses, DNA, Complementary, Fragile X Messenger Ribonucleoprotein 1

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Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

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Fragile X mental retardation protein targets G quartet mRNAs important for neuronal function

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