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Gene Review

Fmr1  -  CG6203 gene product from transcript CG6203-RC

Drosophila melanogaster

Synonyms: AT24755, BcDNA:GM08679, CG6203, Dmel\CG6203, EP(3)3517, ...
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Disease relevance of Fmr1


Psychiatry related information on Fmr1

  • Loss of Fragile X mental retardation protein (FMRP) function causes the highly prevalent Fragile X syndrome [1 and 2] [4].
  • Adult dfmr1 (also called dfxr) mutant flies display arrhythmic circadian activity and have erratic patterns of locomotor activity, whereas overexpression of dFMR1 leads to a lengthened period. dfmr1 mutant males also display reduced courtship activity which appears to result from their inability to maintain courtship interest [5].

High impact information on Fmr1


Biological context of Fmr1

  • We generated specific point mutations or small deletions in the Drosophila fragile X-related (Fmr1) gene and examined the roles of Fmr1 in dendritic development of dendritic arborization (DA) neurons in Drosophila larvae [9].
  • The frequency of central pair microtubule loss becomes progressively greater as spermatogenesis progresses, suggesting that dFXR regulates microtubule stability [2].
  • Male dfxr null mutants have the enlarged testes characteristic of the disease and are nearly sterile (>90% reduced male fecundity). dFXR protein is highly enriched in Drosophila testes, particularly in spermatogenic cells during the early stages of spermatogenesis [2].
  • Interestingly, distinct neuronal cell types show different phenotypes, suggesting that dfxr differentially regulates diverse targets in the brain [10].
  • Fragile X mental retardation is a prominent genetic disorder caused by the lack of the FMR1 gene product, a known RNA binding protein [5].

Anatomical context of Fmr1

  • We found that Fmr1 could be detected in the cell bodies and proximal dendrites of DA neurons and that Fmr1 loss-of-function mutations increased the number of higher-order dendritic branches [9].
  • Taken together, these data show that dFMRP is a potent negative regulator of neuronal architecture and synaptic differentiation in both peripheral and central nervous systems [11].
  • Here, we investigate dFXR function in the testes [2].
  • Ultrastructurally, dfxr mutants lose specifically the central pair microtubules in the sperm tail axoneme [2].
  • We previously established a FraX model in Drosophila, showing that the fly FMRP homologue, dFXR, acts as a negative translational regulator of microtubule-associated Futsch to control stability of the microtubule cytoskeleton during nervous system development [2].

Associations of Fmr1 with chemical compounds

  • They also raise the possibility that compounds having similar effects on metabotropic glutamate receptors may ameliorate cognitive and behavioral defects observed in Fragile X patients [12].
  • The substitutions of conserved isoleucine residues within the KH domains with asparagine are thought to impair binding of RNA substrates and perhaps the ability of FMRP to assemble into mRNP complexes [13].

Regulatory relationships of Fmr1


Other interactions of Fmr1

  • These findings demonstrate that Fmr1 affects dendritic development and that Rac1 is partially responsible for mediating this effect [9].
  • CYFIP mutations affect axons and synapses, much like mutations in dFMR1 (the Drosophila FMR1 ortholog) and in Rho GTPase dRac1 [15].
  • Moreover, Rac1-induced actin remodeling is altered in fibroblasts lacking FMRP or carrying a point-mutation in the KH1 or in the KH2 RNA-binding domain [16].
  • Furthermore, we show that dFMRP associates with endogenous tral mRNA and is required for normal TRAL protein expression and localization, revealing it as a previously undescribed target of dFMRP control [17].
  • In addition, dosage compensation is controlled by Sex-lethal-mediated translational regulation while dFMR1 (the Drosophila homologue of the fragile X mental retardation protein) controls translation of various mRNAs which function in the nervous system [18].

Analytical, diagnostic and therapeutic context of Fmr1

  • Several RNA-binding domains have been identified in FMRP, but the contribution of these individual domains to FMRP function in an animal model is not well understood [13].


  1. Drosophila fragile X-related gene regulates the MAP1B homolog Futsch to control synaptic structure and function. Zhang, Y.Q., Bailey, A.M., Matthies, H.J., Renden, R.B., Smith, M.A., Speese, S.D., Rubin, G.M., Broadie, K. Cell (2001) [Pubmed]
  2. The Drosophila fragile X-related gene regulates axoneme differentiation during spermatogenesis. Zhang, Y.Q., Matthies, H.J., Mancuso, J., Andrews, H.K., Woodruff, E., Friedman, D., Broadie, K. Dev. Biol. (2004) [Pubmed]
  3. A screen for modifiers of decapentaplegic mutant phenotypes identifies lilliputian, the only member of the Fragile-X/Burkitt's Lymphoma family of transcription factors in Drosophila melanogaster. Su, M.A., Wisotzkey, R.G., Newfeld, S.J. Genetics (2001) [Pubmed]
  4. The Drosophila fragile X mental retardation protein controls actin dynamics by directly regulating profilin in the brain. Reeve, S.P., Bassetto, L., Genova, G.K., Kleyner, Y., Leyssen, M., Jackson, F.R., Hassan, B.A. Curr. Biol. (2005) [Pubmed]
  5. Drosophila lacking dfmr1 activity show defects in circadian output and fail to maintain courtship interest. Dockendorff, T.C., Su, H.S., McBride, S.M., Yang, Z., Choi, C.H., Siwicki, K.K., Sehgal, A., Jongens, T.A. Neuron (2002) [Pubmed]
  6. Fragile X-related protein and VIG associate with the RNA interference machinery. Caudy, A.A., Myers, M., Hannon, G.J., Hammond, S.M. Genes Dev. (2002) [Pubmed]
  7. Translational complexity of the fragile x mental retardation protein: insights from the fly. Broadie, K., Pan, L. Mol. Cell (2005) [Pubmed]
  8. Biochemical and genetic interaction between the fragile X mental retardation protein and the microRNA pathway. Jin, P., Zarnescu, D.C., Ceman, S., Nakamoto, M., Mowrey, J., Jongens, T.A., Nelson, D.L., Moses, K., Warren, S.T. Nat. Neurosci. (2004) [Pubmed]
  9. Control of dendritic development by the Drosophila fragile X-related gene involves the small GTPase Rac1. Lee, A., Li, W., Xu, K., Bogert, B.A., Su, K., Gao, F.B. Development (2003) [Pubmed]
  10. Drosophila fragile X protein, DFXR, regulates neuronal morphology and function in the brain. Morales, J., Hiesinger, P.R., Schroeder, A.J., Kume, K., Verstreken, P., Jackson, F.R., Nelson, D.L., Hassan, B.A. Neuron (2002) [Pubmed]
  11. The Drosophila fragile X gene negatively regulates neuronal elaboration and synaptic differentiation. Pan, L., Zhang, Y.Q., Woodruff, E., Broadie, K. Curr. Biol. (2004) [Pubmed]
  12. Pharmacological rescue of synaptic plasticity, courtship behavior, and mushroom body defects in a Drosophila model of fragile X syndrome. McBride, S.M., Choi, C.H., Wang, Y., Liebelt, D., Braunstein, E., Ferreiro, D., Sehgal, A., Siwicki, K.K., Dockendorff, T.C., Nguyen, H.T., McDonald, T.V., Jongens, T.A. Neuron (2005) [Pubmed]
  13. Substitution of Critical Isoleucines in the KH Domains of Drosophila Fragile X Protein Results in Partial Loss-of-Function Phenotypes. Banerjee, P., Nayar, S., Hebbar, S., Fox, C.F., Jacobs, M.C., Park, J.H., Fernandes, J.J., Dockendorff, T.C. Genetics (2007) [Pubmed]
  14. Disruption of the MAP1B-related protein FUTSCH leads to changes in the neuronal cytoskeleton, axonal transport defects, and progressive neurodegeneration in Drosophila. Bettencourt da Cruz, A., Schwärzel, M., Schulze, S., Niyyati, M., Heisenberg, M., Kretzschmar, D. Mol. Biol. Cell (2005) [Pubmed]
  15. CYFIP/Sra-1 controls neuronal connectivity in Drosophila and links the Rac1 GTPase pathway to the fragile X protein. Schenck, A., Bardoni, B., Langmann, C., Harden, N., Mandel, J.L., Giangrande, A. Neuron (2003) [Pubmed]
  16. FMRP interferes with the Rac1 pathway and controls actin cytoskeleton dynamics in murine fibroblasts. Castets, M., Schaeffer, C., Bechara, E., Schenck, A., Khandjian, E.W., Luche, S., Moine, H., Rabilloud, T., Mandel, J.L., Bardoni, B. Hum. Mol. Genet. (2005) [Pubmed]
  17. Fragile X mental retardation protein controls trailer hitch expression and cleavage furrow formation in Drosophila embryos. Monzo, K., Papoulas, O., Cantin, G.T., Wang, Y., Yates, J.R., Sisson, J.C. Proc. Natl. Acad. Sci. U.S.A. (2006) [Pubmed]
  18. Mechanisms of translational regulation in Drosophila. Wilhelm, J.E., Smibert, C.A. Biol. Cell (2005) [Pubmed]
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