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DTor and DFMRP immunoreactivities were partially colocalized in several cellular organelles in larval muscles
Fmr1 protein associates with ninaE (zeige RHO Proteine) mRNA and represses its translation.
Our data strongly support a gain-of-function pathogenic mechanism of PQBP1 (zeige PQBP1 Proteine) 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 (zeige TARDBP Proteine) overexpression
demonstrate that Zfrp8 genetically interacts with Fmr1 and tral (zeige LSM14A Proteine) 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.
upon the stimulation of replication stress, dFMR1 is associated with chromatin in a domain-specific manner, which is essential for its ability to induce the phosphorylation of H2Av (zeige H2AFV Proteine).
APP levels then decrease progressively as a function of age in close relationship with the gradual normalization of FMRP and hnRNP C levels.
FMRP is mostly associated diacylglycerol kinase kappa (Dgkkappa), a master regulator that controls the switch between diacylglycerol and phosphatidic acid signaling pathways.
The tetradecaplex marker assay can be performed directly on single cells or after whole-genome amplification, thus supporting its use in fragile X syndrome preimplantation genetic diagnosis either as a standalone linkage-based assay or as a complement to FMR1 mutation detection.
In the PM group, the lack of any significant association between FMR1 mRNA levels and phenotypic measures found in this study suggests that either FMR1 expression is not well conserved between tissues, or that FMR1 intron 1 methylation is linked to neuroanatomical and cognitive phenotype in PM females via a different mechanism.
Cerebellar and brainstem volumes were likely affected during both development and progression of neurodegeneration in premutation carriers of FMR1.
unmethylated full mutation individuals do not lack the cell-intrinsic ability to silence FMR1 and that inter-individual variability in the CGG repeat size required for silencing exists in the fragile X syndrome population.
CGG-repeat dynamics and FMR1 gene silencing in fragile X syndrome stem cells and stem cell-derived neurons has been reported.
Based on insights from the structures and existing biochemical data, the existence of an evolutionarily conserved ribonucleoprotein (RNP (zeige RNPC3 Proteine)) complex consisting of Caprin-1, FMRP and G3BP1 (zeige G3BP1 Proteine) is proposed.
found that human Torsin1A and human FMRP were present in the same protein complexes, suggesting that this phenomenon is evolutionarily conserved
Understanding how FMRP regulates iontophoresis should reveal new molecular factors underpinning Fragile X syndrome dysfunction
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 (zeige ADAR Proteine) genes and Adar2 (zeige ADARB1 Proteine) 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.
Overexpression of Dgkkappa in neurons is able to rescue the dendritic spine defects of the Fragile X Mental Retardation 1 gene KO neurons.
Results indicate that direct inhibition of acetylcholinesterase (zeige AChE Proteine) activity and up-regulation of m1 muscarinic acetylcholine receptor (zeige CHRNB1 Proteine) expression in the striatum might contribute to the beneficial effects of alpha-asarone on locomotor hyperactivity in Fmr1 knock out mice.
neurogenesis is also accelerated in the embryonic brain of Fmr1-knockout mice, indicating that cellular model recapitulates the molecular alterations present in vivo.
Casein kinase II (zeige CSNK2A1 Proteine) (CK2 (zeige CSNK2A1 Proteine)) phosphorylates murine FMRP S499. Phosphorylation of FMRP S499 permits phosphorylation of additional, nearby residues. Evidence suggests that these nearby residues are modulated by mGluR (zeige GRM8 Proteine)-I and PP2A (zeige PPP2R2B Proteine) pathways; that FMRP is peritranslationally phosphorylated by CK2 (zeige CSNK2A1 Proteine), which allows for secondary phosphorylation of secondary residues in an activity-dependent manner.
FMR1 role in the excitability in hippocampal CA1 (zeige CA1 Proteine) pyramidal cells
Fmr1-KO mice show significantly less daily movement during the dark cycle and more bouts of activity during the light cycle compared with wild types.
these findings support the notion of FMRP differential neuronal regulation and strongly implicate the contribution of fundamental sensory and motor processing at subcortical levels to fragile X syndrome pathology
In Fmr1 KO neurons, Mdm2 (zeige MDM2 Proteine) is hyperphosphorylated, nuclear localized basally, and unaffected by MEF2 (zeige MEF2C Proteine) activation, which our data suggest due to an enhanced interaction with Eukaryotic Elongation Factor (zeige TSFM Proteine) 1alpha (EF1alpha), whose protein levels are elevated in Fmr1 KO. Expression of a dephosphomimetic of Mdm2 (zeige MDM2 Proteine) rescues PSD-95 (zeige DLG4 Proteine) ubiquitination, degradation and synapse elimination in Fmr1 KO neurons.
We show here that in mouse models PFC (zeige CFP Proteine) dysfunction in Fragile X Syndrome (FX) can be attributed to the continued absence of FMRP from the PFC (zeige CFP Proteine), independent of FMRP status during development.
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