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This FMRP activity is mediated solely via a second conserved RNA-binding protein, LIN-28 (show LIN28A Proteins), known to boost insulin (show INS Proteins) signaling in stem cells. Via LIN-28 (show LIN28A Proteins), FMRP controls progenitor cell behavior by post-transcriptionally repressing the level of insulin receptor (InR (show INSR Proteins)).
DTor and DFMRP immunoreactivities were partially colocalized in several cellular organelles in larval muscles
Fmr1 protein associates with ninaE (show RHO Proteins) mRNA and represses its translation.
Our data strongly support a gain-of-function pathogenic mechanism of PQBP1 (show PQBP1 Proteins) c.459_462delAGAG and c.463_464dupAG mutations, and suggest that therapeutic strategies to restore FMRP function may be beneficial for those patients
dFMRP cooperates with Piwi in maintaining genome integrity by silencing heterochromatic genes and suppressing transposon expression.
results show Fragile X Mental Retardation Protein (FMRP) shapes neuron class-specific calcium signaling in excitatory vs. inhibitory neurons in developing learning/memory circuitry, and that FMRP mediates activity-dependent regulation of calcium signaling specifically during the early-use critical period.
results support a model whereby dFMRP can modulate the neurotoxicity caused by TDP-43 (show TARDBP Proteins) overexpression
demonstrate that Zfrp8 genetically interacts with Fmr1 and tral (show LSM14A Proteins) in an antagonistic manner. Zfrp8 and FMRP both control heterochromatin packaging, also in opposite ways
dFmr1 protein is essential for proper cardiac function and establish the fly as a new model for studying the role(s) of FraX proteins in the heart.
These results show that dfmr1 acts in a neuron type-specific activity-dependent manner for sculpting dendritic arbors during early-use, critical period development of learning and memory circuitry in the Drosophila brain.
AKT (show AKT1 Proteins) inhibition led to decreased FMRP levels, as expected due to the known FMR1/FMRP negative feedback loop. But rFSH and the mTOR (show FRAP1 Proteins) inhibition increased them, indicating a decoupling of this FMR1/FMRP negative feedback loop in our model system
The CGG expanded allele of the FMR1 gene might be associated with unexplained multiple miscarriages.
Fragile X-associated disorders encompass several conditions, which are caused by expansion mutations in the fragile X mental retardation 1 (FMR1) gene.
We demonstrate that romidepsin, an inhibitor of class I histone deacetylases, does not activate FMR1 expression in patient cell cultures, whereas vorinostat, an inhibitor of classes I and II histone deacetylases, activates a low level of FMR1 expression in some patient cell lines
the current study shows lower rates of blastocyst development per metaphase II oocyte and 2PN embryos in FMR1 pre-mutation carriers compared to age-matched controls.
Next-generation sequencing in human melanoma cells revealed that FMRP regulates a large number of mRNAs involved in relevant processes of melanoma progression
The naturally occurring Fragile XFMR1 5' region undergoes inactivation post implantation in a Dicer (show DICER1 Proteins)/Ago-dependent targeted process which involves local SUV39H (show SUV39H1 Proteins)-mediated tri (show VANGL2 Proteins)-methylation of histone H3K9. Fragile X syndrome may come about through inadvertent siRNA-mediated heterochromatinization.
The learning disability fragile X syndrome results from the presence of >200 CGG/CCG repeats in exon 1 of the X-linked gene FMR1. Such alleles arise by expansion from maternally transmitted FMR1 premutation alleles, alleles having 55 to 200 repeats. Expansion risk is directly related to maternal repeat number. However, AGG interruptions to the repeat tract are important modifiers of expansion risk.
Women who carry a fragile X premutation, defined as having 55-200 unmethylated CGG repeats in the 5' UTR of the X-linked FMR1 gene, have a 20-fold increased risk for primary ovarian insufficiency .This study supports the idea of a relationship between a fragile X premutation and accelerated biological aging and could be the basis of future studies aimed to better understand the role of this mutation on cellular senescence
Studies indicate the relationship between Fragile X mental retardation 1 (Fmr1) and non-coding RNA pathways, suggesting molecular pathogenesis of Fragile X syndrome, and potential translational applications in clinical management of the disease [Review].
We identified thousands of clustered RNA editing sites in the zebrafish transcriptome and showed that Fmrp biochemically interacts with the Adar2a protein. The expression levels of the adar (show ADAR Proteins) genes and Adar2 (show ADARB1 Proteins) protein increased in fmr1-/- zebrafish
Loss-of-function fmr1 mutants carrying an anti-fmr1 miRNA transgene show abnormal neuronal morphology and connectivity similar to that seen in human fragile X syndrome.
FMRP inhibits ADAR2 (show ADARB1 Proteins) activity, absence of FMRP results in defects of RNA editing of neuronal mRNAs in the mouse model of Fragile X Syndrome.
this study establishes that reducing STEP activity with TC-2153 in Fmr1 KO mice reduces AGS (show GLA Proteins) susceptibility, improves electrophysiologic and synaptic deficits, and normalizes select social and nonsocial anxiety-related behaviors.
Results indicate several sex-specific changes in Fmr1 knockout mice, including male-specific increases in activity levels, and female-specific increases in repetitive behaviors on both the nose-poke assay and motor coordination on the accelerating rotarod task.
Fmr1 regulates stability of Myf5 (show MYF5 Proteins) mRNA and skeletal muscle cell renewal.
The results of this study found decreases in either glycinergic or GABAergic inhibition to the medial nucleus of the trapezoid body (MNTB) specific to the tonotopic location within the nucleus in Fmr1 knockout mice.
mGluR5 (show GRM5 Proteins) was significantly more mobile at synapses in hippocampal Fmr1 KO neurons, causing an increased synaptic surface co-clustering of mGluR5 (show GRM5 Proteins) and NMDAR (show GRIN1 Proteins).
The results show that SMNDC1 (show SMNDC1 Proteins) mRNA 5'-UTR forms an intramolecular, parallel G quadruplex structure comprised of three G quartet planes, which is bound specifically by FMRP both in vitro and in mouse brain lysates.
Using the Fmr1 null mouse model of fragile X syndrome, brain regions, gene networks, and molecular pathways responsive to a social stimulus have been identified.
This study demonstrated that In vivo recordings from barrel cortex revealed that Fmr1 KO mice show an enlargement in the cortical area activated by whisker deflections.
The protein encoded by this gene binds RNA and is associated with polysomes. The encoded protein may be involved in mRNA trafficking from the nucleus to the cytoplasm. A trinucleotide repeat (CGG) in the 5' UTR is normally found at 6-53 copies, but an expansion to 55-230 repeats is the cause of fragile X syndrome. Expansion of the trinucleotide repeat may also cause one form of premature ovarian failure (POF1). Multiple alternatively spliced transcript variants that encode different protein isoforms and which are located in different cellular locations have been described for this gene.
, Fragile-X mental retardation protein
, drosophila fragile X mental retardation protein
, fragile X
, fragile X mental retardation
, fragile X mental retardation 1
, fragile X mental retardation gene
, fragile X mental retardation protein
, fragile X protein
, fragile X related protein
, fragile X-related
, fragile x related
, fragile X mental retardation protein 1
, fragile X mental retardation protein 1 homolog
, fragile X mental retardation syndrome 1 homolog
, fragile X mental retardation-1 protein
, protein FMR-1
, ragile X mental retardation protein
, fragile X mental retardation 1 protein