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Fragile X messenger ribonucleoprotein 1

Definition:

Multifunctional polyribosome-associated RNA-binding protein that plays a central role in neuronal development and synaptic plasticity through the regulation of alternative mRNA splicing, mRNA stability, mRNA dendritic transport and postsynaptic local protein synthesis of a subset of mRNAs. Plays a role in the alternative splicing of its own mRNA. Plays a role in mRNA nuclear export (By similarity). Together with export factor NXF2, is involved in the regulation of the NXF1 mRNA stability in neurons (By similarity). Stabilizes the scaffolding postsynaptic density protein DLG4/PSD-95 and the myelin basic protein (MBP) mRNAs in hippocampal neurons and glial cells, respectively; this stabilization is further increased in response to metabotropic glutamate receptor (mGluR) stimulation (By similarity). Plays a role in selective delivery of a subset of dendritic mRNAs to synaptic sites in response to mGluR activation in a kinesin-dependent manner (By similarity). Plays a role as a repressor of mRNA translation during the transport of dendritic mRNAs to postsynaptic dendritic spines. Component of the CYFIP1-EIF4E-FMR1 complex which blocks cap-dependent mRNA translation initiation (By similarity). Represses mRNA translation by stalling ribosomal translocation during elongation (By similarity). Reports are contradictory with regards to its ability to mediate translation inhibition of MBP mRNA in oligodendrocytes. Also involved in the recruitment of the RNA helicase MOV10 to a subset of mRNAs and hence regulates microRNA (miRNA)-mediated translational repression by AGO2. Facilitates the assembly of miRNAs on specific target mRNAs. Also plays a role as an activator of mRNA translation of a subset of dendritic mRNAs at synapses. In response to mGluR stimulation, FMR1-target mRNAs are rapidly derepressed, allowing for local translation at synapses (By similarity). Binds to a large subset of dendritic mRNAs that encode a myriad of proteins involved in pre- and postsynaptic functions. Binds to 5'-ACU[GU]-3' and/or 5'-[AU]GGA-3' RNA consensus sequences within mRNA targets, mainly at coding sequence (CDS) and 3'-untranslated region (UTR) and less frequently at 5'-UTR. Binds to intramolecular G-quadruplex structures in the 5'- or 3'-UTRs of mRNA targets. Binds to G-quadruplex structures in the 3'-UTR of its own mRNA. Binds also to RNA ligands harboring a kissing complex (kc) structure; this binding may mediate the association of FMR1 with polyribosomes. Binds mRNAs containing U-rich target sequences. Binds to a triple stem-loop RNA structure, called Sod1 stem loop interacting with FMRP (SoSLIP), in the 5'-UTR region of superoxide dismutase SOD1 mRNA. Binds to the dendritic, small non-coding brain cytoplasmic RNA 1 (BC1); which may increase the association of the CYFIP1-EIF4E-FMR1 complex to FMR1 target mRNAs at synapses (By similarity). Associates with export factor NXF1 mRNA-containing ribonucleoprotein particles (mRNPs) in a NXF2-dependent manner (By similarity). Binds to a subset of miRNAs in the brain. May associate with nascent transcripts in a nuclear protein NXF1-dependent manner. In vitro, binds to RNA homomer; preferentially on poly(G) and to a lesser extent on poly(U), but not on poly(A) or poly(C). Moreover, plays a role in the modulation of the sodium-activated potassium channel KCNT1 gating activity. Negatively regulates the voltage-dependent calcium channel current density in soma and presynaptic terminals of dorsal root ganglion (DRG) neurons, and hence regulates synaptic vesicle exocytosis (By similarity). Modulates the voltage-dependent calcium channel CACNA1B expression at the plasma membrane by targeting the channels for proteosomal degradation (By similarity). Plays a role in regulation of MAP1B-dependent microtubule dynamics during neuronal development (By similarity). Recently, has been shown to play a translation-independent role in the modulation of presynaptic action potential (AP) duration and neurotransmitter release via large-conductance calcium-activated potassium (BK) channels in hippocampal and cortical excitatory neurons. Finally, FMR1 may be involved in the control of DNA damage response (DDR) mechanisms through the regulation of ATR-dependent signaling pathways such as histone H2AX/H2A.x and BRCA1 phosphorylations.; [Isoform 10]: Binds to RNA homomer; preferentially on poly(G) and to a lesser extent on poly(U), but not on poly(A) or poly(C). May bind to RNA in Cajal bodies.; [Isoform 6]: Binds to RNA homomer; preferentially on poly(G) and to a lesser extent on poly(U), but not on poly(A) or poly(C). May bind to RNA in Cajal bodies.; (Microbial infection) Acts as a positive regulator of influenza A virus (IAV) replication. Required for the assembly and nuclear export of the viral ribonucleoprotein (vRNP) components.

References:

[1]. Leila K Myrick, et al. Fragile X syndrome due to a missense mutation. Eur J Hum Genet. 2014 Oct;22(10):1185-9. [Content Brief]

[2]. M C Siomi, et al. FXR1, an autosomal homolog of the fragile X mental retardation gene. EMBO J. 1995 Jun 1;14(11):2401-8. [Content Brief]

[3]. Anthony Giampetruzzi, et al. FMRP and myelin protein expression in oligodendrocytes. Mol Cell Neurosci. 2013 Sep;56:333-41. [Content Brief]

[4]. Miri Kim, et al. Fragile X mental retardation protein FMRP binds mRNAs in the nucleus. Mol Cell Biol. 2009 Jan;29(1):214-28. [Content Brief]

[5]. C T Ashley Jr, et al. FMR1 protein: conserved RNP family domains and selective RNA binding. Science. 1993 Oct 22;262(5133):563-6. [Content Brief]

[6]. Alain Y Dury, et al. Nuclear Fragile X Mental Retardation Protein is localized to Cajal bodies. PLoS Genet. 2013 Oct;9(10):e1003890. [Content Brief]

[7]. Manuel Ascano Jr, et al. FMRP targets distinct mRNA sequence elements to regulate protein expression. Nature. 2012 Dec 20;492(7429):382-6. [Content Brief]

[8]. Phillip J Kenny, et al. MOV10 and FMRP regulate AGO2 association with microRNA recognition elements. Cell Rep. 2014 Dec 11;9(5):1729-1741. [Content Brief]

[9]. Zhuo Zhou, et al. Fragile X mental retardation protein stimulates ribonucleoprotein assembly of influenza A virus. Nat Commun. 2014;5:3259. [Content Brief]

[10]. H Siomi, et al. The protein product of the fragile X gene, FMR1, has characteristics of an RNA-binding protein. Cell. 1993 Jul 30;74(2):291-8. [Content Brief]

[11]. Elias G Bechara, et al. A novel function for fragile X mental retardation protein in translational activation. PLoS Biol. 2009 Jan 20;7(1):e16. [Content Brief]

[12]. Yang Zhang, et al. FMRP interacts with G-quadruplex structures in the 3'-UTR of its dendritic target Shank1 mRNA. RNA Biol. 2014;11(11):1364-74. [Content Brief]

[13]. J C Darnell, et al. Fragile X mental retardation protein targets G quartet mRNAs important for neuronal function. Cell. 2001 Nov 16;107(4):489-99. [Content Brief]

[14]. Lakshmi Menon, et al. Fragile X mental retardation protein interactions with the microtubule associated protein 1B RNA. RNA. 2008 Aug;14(8):1644-55. [Content Brief]

[15]. Oliver Rackham, et al. Visualization of RNA-protein interactions in living cells: FMRP and IMP1 interact on mRNAs. EMBO J. 2004 Aug 18;23(16):3346-55. [Content Brief]

[16]. B Laggerbauer, et al. Evidence that fragile X mental retardation protein is a negative regulator of translation. Hum Mol Genet. 2001 Feb 15;10(4):329-38. [Content Brief]

[17]. C Schaeffer, et al. The fragile X mental retardation protein binds specifically to its mRNA via a purine quartet motif. EMBO J. 2001 Sep 3;20(17):4803-13. [Content Brief]

[18]. Roman Alpatov, et al. A chromatin-dependent role of the fragile X mental retardation protein FMRP in the DNA damage response. Cell. 2014 May 8;157(4):869-81. [Content Brief]

[19]. Francesca Zalfa, et al. A new function for the fragile X mental retardation protein in regulation of PSD-95 mRNA stability. Nat Neurosci. 2007 May;10(5):578-87. [Content Brief]

[20]. Maile R Brown, et al. Fragile X mental retardation protein controls gating of the sodium-activated potassium channel Slack. Nat Neurosci. 2010 Jul;13(7):819-21. [Content Brief]

[21]. Peng Jin, et al. Biochemical and genetic interaction between the fragile X mental retardation protein and the microRNA pathway. Nat Neurosci. 2004 Feb;7(2):113-7. [Content Brief]

[22]. Marie-Cécile Didiot, et al. The G-quartet containing FMRP binding site in FMR1 mRNA is a potent exonic splicing enhancer. Nucleic Acids Res. 2008 Sep;36(15):4902-12. [Content Brief]

[23]. L Chen, et al. The fragile X mental retardation protein binds and regulates a novel class of mRNAs containing U rich target sequences. Neuroscience. 2003;120(4):1005-17. [Content Brief]

[24]. Michael Fähling, et al. Translational regulation of the human achaete-scute homologue-1 by fragile X mental retardation protein. J Biol Chem. 2009 Feb 13;284(7):4255-66. [Content Brief]

[25]. Isabelle Plante, et al. Dicer-derived microRNAs are utilized by the fragile X mental retardation protein for assembly on target RNAs. J Biomed Biotechnol. 2006;2006(4):64347. [Content Brief]

[26]. H Siomi, et al. 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. Cell. 1994 Apr 8;77(1):33-9. [Content Brief]

[27]. S Adinolfi, et al. The N-terminus of the fragile X mental retardation protein contains a novel domain involved in dimerization and RNA binding. Biochemistry. 2003 Sep 9;42(35):10437-44. [Content Brief]

[28]. Jennifer C Darnell, et al. Kissing complex RNAs mediate interaction between the Fragile-X mental retardation protein KH2 domain and brain polyribosomes. Genes Dev. 2005 Apr 15;19(8):903-18. [Content Brief]

[29]. Laura N Antar, et al. Local functions for FMRP in axon growth cone motility and activity-dependent regulation of filopodia and spine synapses. Mol Cell Neurosci. 2006 May-Jun;32(1-2):37-48. [Content Brief]

[30]. Ying Ju Sung, et al. The fragile X mental retardation protein FMRP binds elongation factor 1A mRNA and negatively regulates its translation in vivo. J Biol Chem. 2003 May 2;278(18):15669-78. [Content Brief]

[31]. Rajesh P Menon, et al. The C terminus of fragile X mental retardation protein interacts with the multi-domain Ran-binding protein in the microtubule-organising centre. J Mol Biol. 2004 Oct 8;343(1):43-53. [Content Brief]

[32]. Leila K Myrick, et al. Independent role for presynaptic FMRP revealed by an FMR1 missense mutation associated with intellectual disability and seizures. Proc Natl Acad Sci U S A. 2015 Jan 27;112(4):949-56. [Content Brief]

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