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Fragile X syndrome is caused by the absence of protein FMRP, the function of which is still poorly understood. Previous studies have suggested that FMRP may be involved in various aspects of mRNA metabolism, including transport, stability and/or translatability. FMRP was shown to interact with a subset of brain mRNAs as well as with its own mRNA; however, no specific RNA-binding site could be identified precisely. Here, we report the identification and characterization of a specific and high affinity binding site for FMRP in the RGG-coding region of its own mRNA. This site contains a purine quartet motif that is essential for FMRP binding and can be substituted by a heterologous quartet-forming motif. The specific binding of FMRP to its target site was confirmed further in a reticulocyte lysate through its ability to repress translation of a reporter gene harboring the RNA target site in the 5'-untranslated region. Our data address interesting questions concerning the role of FMRP in the post-transcriptional control of its own gene and possibly other target genes.
Ashley,
FMR1 protein: conserved RNP family domains and selective RNA binding.
1993, Pubmed
Ashley,
FMR1 protein: conserved RNP family domains and selective RNA binding.
1993,
Pubmed Ashley,
Human and murine FMR-1: alternative splicing and translational initiation downstream of the CGG-repeat.
1993,
Pubmed Bardoni,
A novel RNA-binding nuclear protein that interacts with the fragile X mental retardation (FMR1) protein.
1999,
Pubmed Bashkirov,
A mouse cytoplasmic exoribonuclease (mXRN1p) with preference for G4 tetraplex substrates.
1997,
Pubmed Brown,
Purified recombinant Fmrp exhibits selective RNA binding as an intrinsic property of the fragile X mental retardation protein.
1998,
Pubmed Brunel,
Probing RNA structure and RNA-ligand complexes with chemical probes.
2000,
Pubmed Ceman,
Isolation of an FMRP-associated messenger ribonucleoprotein particle and identification of nucleolin and the fragile X-related proteins as components of the complex.
1999,
Pubmed Cheong,
Solution structure of an unusually stable RNA tetraplex containing G- and U-quartet structures.
1992,
Pubmed Christiansen,
A guanosine quadruplex and two stable hairpins flank a major cleavage site in insulin-like growth factor II mRNA.
1994,
Pubmed Chu,
Identification of an RNA binding site for human thymidylate synthase.
1993,
Pubmed Comery,
Abnormal dendritic spines in fragile X knockout mice: maturation and pruning deficits.
1997,
Pubmed Corbin,
The fragile X mental retardation protein is associated with poly(A)+ mRNA in actively translating polyribosomes.
1997,
Pubmed Devys,
The FMR-1 protein is cytoplasmic, most abundant in neurons and appears normal in carriers of a fragile X premutation.
1993,
Pubmed Dreyfuss,
hnRNP proteins and the biogenesis of mRNA.
1993,
Pubmed Eberhart,
The fragile X mental retardation protein is a ribonucleoprotein containing both nuclear localization and nuclear export signals.
1996,
Pubmed Ercikan-Abali,
Dihydrofolate reductase protein inhibits its own translation by binding to dihydrofolate reductase mRNA sequences within the coding region.
1997,
Pubmed Feng,
FMRP associates with polyribosomes as an mRNP, and the I304N mutation of severe fragile X syndrome abolishes this association.
1997,
Pubmed Fry,
The fragile X syndrome d(CGG)n nucleotide repeats form a stable tetrahelical structure.
1994,
Pubmed Fu,
Participation of the human p53 3'UTR in translational repression and activation following gamma-irradiation.
1997,
Pubmed Fujinaga,
Mechanism of carcinogenesis by RNA tumor viruses. 3. Formation of RNA, DNA complex and duplex DNA molecules by the DNA polymerase (s) of avian myeloblastosis virus.
1970,
Pubmed Gibson,
KH domains within the FMR1 sequence suggest that fragile X syndrome stems from a defect in RNA metabolism.
1993,
Pubmed Gray,
Iron regulatory protein prevents binding of the 43S translation pre-initiation complex to ferritin and eALAS mRNAs.
1994,
Pubmed Hamilton,
Modulation of AUUUA response element binding by heterogeneous nuclear ribonucleoprotein A1 in human T lymphocytes. The roles of cytoplasmic location, transcription, and phosphorylation.
1997,
Pubmed Jin,
Understanding the molecular basis of fragile X syndrome.
2000,
Pubmed Jing,
Mechanism of inhibition of HIV-1 integrase by G-tetrad-forming oligonucleotides in Vitro.
2000,
Pubmed Kang,
Crystal structure of four-stranded Oxytricha telomeric DNA.
1992,
Pubmed Katahira,
RNA quadruplex containing G and A.
1995,
Pubmed Kim,
Tetramerization of an RNA oligonucleotide containing a GGGG sequence.
1991,
Pubmed Kozak,
Influences of mRNA secondary structure on initiation by eukaryotic ribosomes.
1986,
Pubmed Kozak,
Circumstances and mechanisms of inhibition of translation by secondary structure in eucaryotic mRNAs.
1989,
Pubmed Laggerbauer,
Evidence that fragile X mental retardation protein is a negative regulator of translation.
2001,
Pubmed
,
Xenbase Laughlan,
The high-resolution crystal structure of a parallel-stranded guanine tetraplex.
1994,
Pubmed Lee,
The stability of polypurine tetraplexes in the presence of mono- and divalent cations.
1990,
Pubmed Li,
The fragile X mental retardation protein inhibits translation via interacting with mRNA.
2001,
Pubmed Lin,
Characterization of a cis-acting regulatory element in the protein coding region of thymidylate synthase mRNA.
2000,
Pubmed Liu,
Gene disruption of a G4-DNA-dependent nuclease in yeast leads to cellular senescence and telomere shortening.
1995,
Pubmed Muhlrad,
Turnover mechanisms of the stable yeast PGK1 mRNA.
1995,
Pubmed Oliver,
Preferential binding of fd gene 5 protein to tetraplex nucleic acid structures.
2000,
Pubmed Pitulle,
Phylogeny of rapidly growing members of the genus Mycobacterium.
1992,
Pubmed Rangan,
Induction of duplex to G-quadruplex transition in the c-myc promoter region by a small molecule.
2001,
Pubmed Romby,
The expression of E.coli threonyl-tRNA synthetase is regulated at the translational level by symmetrical operator-repressor interactions.
1996,
Pubmed Schenck,
A highly conserved protein family interacting with the fragile X mental retardation protein (FMRP) and displaying selective interactions with FMRP-related proteins FXR1P and FXR2P.
2001,
Pubmed Scheper,
Long-range RNA interaction of two sequence elements required for endonucleolytic cleavage of human insulin-like growth factor II mRNAs.
1995,
Pubmed Sen,
A sodium-potassium switch in the formation of four-stranded G4-DNA.
1990,
Pubmed Simonsson,
DNA tetraplex formation in the control region of c-myc.
1998,
Pubmed Siomi,
Essential role for KH domains in RNA binding: impaired RNA binding by a mutation in the KH domain of FMR1 that causes fragile X syndrome.
1994,
Pubmed Siomi,
Specific sequences in the fragile X syndrome protein FMR1 and the FXR proteins mediate their binding to 60S ribosomal subunits and the interactions among them.
1996,
Pubmed Sittler,
Alternative splicing of exon 14 determines nuclear or cytoplasmic localisation of fmr1 protein isoforms.
1996,
Pubmed Smith,
Quadruplex structure of Oxytricha telomeric DNA oligonucleotides.
1992,
Pubmed Sundquist,
Telomeric DNA dimerizes by formation of guanine tetrads between hairpin loops.
1989,
Pubmed Sundquist,
Evidence for interstrand quadruplex formation in the dimerization of human immunodeficiency virus 1 genomic RNA.
1993,
Pubmed Sung,
RNAs that interact with the fragile X syndrome RNA binding protein FMRP.
2000,
Pubmed Svitkin,
General RNA binding proteins render translation cap dependent.
1996,
Pubmed Tamanini,
Different targets for the fragile X-related proteins revealed by their distinct nuclear localizations.
1999,
Pubmed Wan,
Characterization of dFMR1, a Drosophila melanogaster homolog of the fragile X mental retardation protein.
2000,
Pubmed Weiler,
Fragile X mental retardation protein is translated near synapses in response to neurotransmitter activation.
1997,
Pubmed Wells,
Circularization of mRNA by eukaryotic translation initiation factors.
1998,
Pubmed Williamson,
Monovalent cation-induced structure of telomeric DNA: the G-quartet model.
1989,
Pubmed Zahler,
Inhibition of telomerase by G-quartet DNA structures.
1991,
Pubmed Zhang,
The fragile X mental retardation syndrome protein interacts with novel homologs FXR1 and FXR2.
1995,
Pubmed
