Recombinant Drosophila melanogaster Fragile X mental retardation syndrome-related protein 1 (Fmr1), partial

1. Stored oocytes lacking FMR1 usually generate embryos with severe neural defects, unlike stored wild-type oocytes, which suggests that translation of multiple large proteins by stored mRNAs is defective in fragile X syndrome and possibly other autism spectrum disorders. PMID: 30115809
2. This FMRP activity is mediated solely via a second conserved RNA-binding protein, LIN-28, known to boost insulin signaling in stem cells. Via LIN-28, FMRP controls progenitor cell behavior by post-transcriptionally repressing the level of insulin receptor (InR). PMID: 29212015
3. DTor and DFMRP immunoreactivities were partially colocalized in several cellular organelles in larval muscles PMID: 27313903
4. Synaptic release of neuropeptides and neurotrophins depends on presynaptic accumulation of dense-core vesicles (DCVs). At rest, DCVs are captured bidirectionally as they circulate through Drosophila motoneuron terminals by anterograde and retrograde transport. Here we show that activity stimulates further synaptic capture that is distinguished from basal capture by its selectivity for anterograde DCVs and its inhibition b PMID: 27852784
5. Fmr1 protein associates with ninaE mRNA and represses its translation. PMID: 27702760
6. Our data strongly support a gain-of-function pathogenic mechanism of PQBP1 c.459_462delAGAG and c.463_464dupAG mutations, and suggest that therapeutic strategies to restore FMRP function may be beneficial for those patients PMID: 28073926
7. 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. PMID: 26851502
8. results support a model whereby dFMRP can modulate the neurotoxicity caused by TDP-43 overexpression PMID: 26385636
9. demonstrate that Zfrp8 genetically interacts with Fmr1 and tral in an antagonistic manner. Zfrp8 and FMRP both control heterochromatin packaging, also in opposite ways PMID: 26772998
10. 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. PMID: 26571124
11. Driving expression of FMRpolyG enhanced induction of UPS impairment in cell models, while prevention of RAN translation attenuated UPS impairment in cells and suppressed the genetic interaction with UPS manipulation in Drosophila PMID: 25954027
12. 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. PMID: 25804740
13. 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. PMID: 24833720
14. The results of this study show that GABAergic circuit structure and function are impaired in the FXS disease state PMID: 24423648
15. FMRP inhibits translation by binding directly to the L5 protein on the 80S ribosome. PMID: 24746697
16. FMRP negatively regulates HSPG co-receptors controlling trans-synaptic signaling during synaptogenesis PMID: 24046358
17. FMRP and Ataxin-2 have roles in long-term olfactory habituation and neuronal translational control PMID: 24344294
18. Dfmr1 mutants are less active than wild-type flies and interact with each other less often. Results raise the possibility of defective social expression with preserved receptive abilities. PMID: 20519966
19. Lig regulates cell proliferation during development in concert with Rin, Capr and FMR1. PMID: 23874212
20. studies thus suggest that the mechanisms controlling the recruitment of FMRP into SG and those that promote its shuttling between granules and the cytosol are uncoupled PMID: 23408971
21. FMRP is required in vivo, in glia for neuroblast reactivation and suggest that it may do so by regulating the output of the insulin signaling pathway PMID: 22513101
22. Study identified a number of mRNAs and miRNAs (miRNA-1 and miRNA-281) with circadian rhythm-dependent altered expression in dfmr1 mutant flies. PMID: 22655085
23. Fmr1 encodes two functional isoforms with respect to expression and activity throughout neuronal development. PMID: 21333716
24. The results of this study suggested that dFMR1 with the RNA-editing pathway and suggest that proper NMJ synaptic architecture requires modulation of dADAR activity by dFMR1. PMID: 22037499
25. requirement of dFMRP for the retention of transitory peptidergic neurons shows evident circuit specificity PMID: 21596027
26. Changes in magnitude and fidelity of calcium signals in the absence of FMRP may contribute to defects in neuronal structure and function, leading to hallmark learning and memory dysfunction of fragile X syndrome. PMID: 20843478
27. study demonstrates that the gene Fmr1 plays an important role in sleep-dependent synaptic renormalization PMID: 21700878
28. These results demonstrate that dFMRP plays a crucial role in controlling MT formation and mitochondrial transport. PMID: 20935173
29. Data show that in the early embryo dFMRP associates specifically with Caprin, and with eIF4G, a key regulator of translational initiation. PMID: 21068064
30. FMR1 protein expression in blood smear for Fragile X syndrome diagnosis. PMID: 20642356
31. FMRP is required during brain development to control the exit from quiescence and proliferative capacity of neuroblasts as well as neuron production. PMID: 20504994
32. These results indicate that FMR1 gene function is evolutionarily conserved in neural mechanisms and cannot be compensated by either FXR1 or FXR2, but that all three proteins can substitute for each other in non-neuronal requirements. PMID: 20442204
33. A family with one patient with mild mental retardation who showed an atypical profile at Southern analysis due to the -413C > G transversion located in the FMR1 promoter, is presented. PMID: 19836446
34. two isoforms of dFMR1 have differential roles in mediating neural development and behavior functions conferred by the dfmr1 gene; Expression of both isoforms is necessary for optimal performance in short- and long-term memory of courtship training PMID: 20463240
35. The clinical and genetic characterization of a new X-linked mental retardation syndrome characterized by short stature, hypogonadism and facial dysmorphism caused by a small Xq27.3q28 interstitial duplication encompassing the FMR1 gene, is reported. PMID: 19844254
36. we show that PRMT1 methylates FMRP in cells, suggesting a model where methylation of the RGG box modulates either the quantity or the identity of the RNAs bound by FMRP. PMID: 20064924
37. Argonaute2 suppresses Drosophila fragile X expression preventing neurogenesis and oogenesis defects PMID: 19888420
38. FMR1 regulates normal neurite extension, guidance and branching. PMID: 12086643
39. FMR1 mutants display arrhythmic circadian activity and locomotor activity; and males display reduced courtship interest. PMID: 12086644
40. a role for the Drosophila fragile X-related gene in circadian output; loss-of-function mutation affects circadian behavior PMID: 12176363
41. have isolated a dFMR1-associated complex that includes two ribosomal proteins, L5 and L11, along with 5S RNA. The dFMR1 complex also contains Argonaute2 (AGO2) and a Drosophila homolog of p68 RNA helicase (Dmp68) PMID: 12368261
42. casein kinase II directly interacts with and phosphorylates dFMR1 in vitro; studies support the model that the biological functions of FMR1, such as regulation of gene expression, are likely regulated by its phosphorylation PMID: 12446764
43. dFMR1 is subjected to transcriptional and posttranscriptional regulation during development, it homomerizes, and its expression recapitulates that of the human FXR protein family: it is enriched in muscles, in central nervous system and in gonads PMID: 12460546
44. Fmr1 affects dendritic development and Rac1 is partially responsible for mediating this effect. PMID: 14530299
45. dFXR regulates microtubule stability in both synaptogenesis in the nervous system and spermatogenesis in the testes. PMID: 15183715
46. demonstrate specific developmental defects of mushroom bodies morphogenesis in dfmr1 mutants that are modulated by genetic interactions PMID: 15215302
47. dFMRP is a potent negative regulator of neuronal architecture and synaptic differentiation in both peripheral and central nervous systems. PMID: 15498496
48. dFMRP normally down-regulates the monoamine pathway, which is consequently up-regulated in the mutant condition PMID: 15634690
49. DFMR1 functions as a negative regulator in the orb autoregulatory circuit, downregulating orb mRNA translation. PMID: 15737929
50. regulation of the actin cytoskeleton is under FMRP1 control. FMRP1 binds the mRNA of the Drosophila profilin and negatively regulates Profilin protein expression PMID: 15964283

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KEGG: dme:Dmel_CG6203

STRING: 7227.FBpp0300445

UniGene: Dm.5279