• Human FMR1 (Fragile X Mental Retardation 1) Sandwich ELISA Kit Ready-To-Use (STJE0015329)

Human FMR1 (Fragile X Mental Retardation 1) Sandwich ELISA Kit Ready-To-Use (STJE0015329)

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STJE0015329

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Applications: ELISA
Reactivity: Human
Note: STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS.
Sensitivity: 0.105ng/mL
Detection Limit: 0.312-20ng/mL
Short Description: This FMR1 Sandwich ELISA Kit, Ready-To-Use is an in-vitro enzyme-linked immunosorbent assay for the measurement of samples in human cell culture supernatant, serum and plasma (EDTA, citrate, heparin).
Storage Instruction: The whole kit may be stored at-20°C for up to 12 months from receipt. An unopened kit may be stored in the fridge at 2-8°C for up to 6 months. Once opened store individual kit contents according to components table provided with the kit.
Assay Time: 3 hrs
Gene Symbol: FMR1
Gene ID: 2332
Uniprot ID: FMR1_HUMAN
Immunogen Region: Ready-To-Use
Sample Type: tissue homogenates, cell lysates or other biological fluids.
Tissue Specificity Expressed in the brain, cerebellum and testis. Also expressed in epithelial tissues. Expressed in mature oligodendrocytes (OLGs). Expressed in fibroblast. Expressed in neurons, Purkinje cells and spermatogonias (at protein level). Expressed in brain, testis and placenta. Expressed in neurons and lymphocytes.
Post Translational Modifications Phosphorylated on several serine residues. Phosphorylation by casein kinase II (CK2) promotes interaction with CAPRIN1 and liquid-liquid phase separation (LLPS) for the formation of a membraneless compartment that concentrates mRNAs with associated regulatory factors. Phosphorylation at Ser-500 by CK2 promotes secondary phosphorylation of other nearby serine residues. Phosphorylation has no effect on the binding of individual mRNA species. Unphosphorylated FMR1 is associated with actively translating polyribosome, whereas a fraction of phosphorylated FMR1 is associated with apparently stalled polyribosome. Dephosphorylation by an activated phosphatase may release the FMR1-mediated translational repression and allow synthesis of a locally required protein at synapses. Monoubiquitinated. Polyubiquitinated. Ubiquitinated and targeted for proteasomal degradation after activation of metabotropic glutamate receptor (mGluR). Monomethylated and asymmetrically dimethylated by PRMT1, PRMT3 and PRMT4 at four arginine residues of the arginine-glycine-glycine box. Methylation decreases ability to undergo liquid-liquid phase separation (LLPS) for the formation of a membraneless compartment. Methylation does not affect mRNA-binding. Methylation is necessary for heterodimerization with FXR1, association with polyribosomes, recruitment into stress granules and translation of FMR1 target mRNAs. Isoform 10: Undergoes proteolytic cleavage.may be specifically cleaved by calpain-1/CAPN1 in cajal bodies.
Function 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 target mRNAs. Acts as an mRNA regulator by mediating formation of some phase-separated membraneless compartment: undergoes liquid-liquid phase separation upon binding to target mRNAs, leading to assemble mRNAs into cytoplasmic ribonucleoprotein granules that concentrate mRNAs with associated regulatory factors. Plays a role in the alternative splicing of its own mRNA. 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. 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. Undergoes liquid-liquid phase separation following phosphorylation and interaction with CAPRIN1, promoting formation of cytoplasmic ribonucleoprotein granules that concentrate mRNAs with factors that inhibit translation and mediate deadenylation of target mRNAs. Acts as a repressor of mRNA translation in synaptic regions by mediating formation of neuronal ribonucleoprotein granules and promoting recruitmtent of EIF4EBP2. 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. Represses mRNA translation by stalling ribosomal translocation during elongation. 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. Binds to a large subset of dendritic mRNAs that encode a myriad of proteins involved in pre- and postsynaptic functions. Binds to 5'-ACUGU-3' and/or 5'-AUGGA-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. Plays a role in mRNA nuclear export. Specifically recognizes and binds a subset of N6-methyladenosine (m6A)-containing mRNAs, promoting their nuclear export in a XPO1/CRM1-dependent manner. Together with export factor NXF2, is involved in the regulation of the NXF1 mRNA stability in neurons. Associates with export factor NXF1 mRNA-containing ribonucleoprotein particles (mRNPs) in a NXF2-dependent manner. 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. Modulates the voltage-dependent calcium channel CACNA1B expression at the plasma membrane by targeting the channels for proteasomal degradation. Plays a role in regulation of MAP1B-dependent microtubule dynamics during neuronal development. 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.
Protein Name Fragile X Messenger Ribonucleoprotein 1
Fragile X Messenger Ribonucleoprotein
Fmrp
Protein Fmr-1
Database Links
Cellular Localisation Cytoplasm
Cytoplasmic Ribonucleoprotein Granule
Stress Granule
Perikaryon
Perinuclear Region
Cell Projection
Neuron Projection
Axon
Dendrite
Dendritic Spine
Synapse
Synaptosome
Growth Cone
Filopodium Tip
Postsynaptic Cell Membrane
Presynaptic Cell Membrane
Nucleus
Nucleolus
Chromosome
Centromere
Cell Membrane
Mediates Formation And Localizes To Cytoplasmic Ribonucleoprotein Membraneless Compartments
Localizes To Cytoplasmic Ribonucleoprotein Granules
Also Referred To As Messenger Ribonucleoprotein Particles Or Mrnps
Along Dendrites And Dendritic Spines
Fmr1-Containing Cytoplasmic Granules Colocalize To F-Actin-Rich Structures
Including Filopodium
Spines And Growth Cone During The Development Of Hippocampal Neurons
Fmr1-Containing Cytoplasmic Granules Are Transported Out Of The Soma Along Axon And Dendrite To Synaptic Contacts In A Microtubule- And Kinesin-Dependent Manner
Colocalizes With Fxr1 And Fxr2 In Discrete Granules
Called Fragile X Granules (Fxgs)
Along Axon And Presynaptic Compartments
Colocalizes With Tdrd3 In Cytoplasmic Stress Granules (Sgs) In Response To Various Cellular Stress
Colocalizes With Fxr1
Kinesin
60s Acidic Ribosomal Protein Rplp0 And Smn In Cytoplasmic Granules In The Soma And Neurite Cell Processes
Colocalizes With H2ax/H2a
X In Pericentromeric Heterochromatin In Response To Dna Damaging Agents
Localizes On Meiotic Pachytene-Stage Chromosomes
Forms Nuclear Foci Representing Sites Of Ongoing Dna Replication In Response To Dna Damaging Agents
Shuttles Between Nucleus And Cytoplasm In A Xpo1/Crm1-Dependent Manner
Colocalizes With Cacna1b In The Cytoplasm And At The Cell Membrane Of Neurons
Colocalizes With Cyfip1
Cyfip2
Nxf2 And Ribosomes In The Perinuclear Region
Colocalizes With Cyfip1 And Eif4e In Dendrites And Probably At Synapses
Isoform 6: Cytoplasm
Isoform 9: Cytoplasm
Isoform 10: Nucleus
Cajal Body
Colocalizes With Colin And Smn In Cajal Bodies
Isoform 11: Nucleus
Alternative ELISA Names Fragile X Messenger Ribonucleoprotein 1 ELISA kit
Fragile X Messenger Ribonucleoprotein ELISA kit
Fmrp ELISA kit
Protein Fmr-1 ELISA kit
FMR1 ELISA kit
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Information sourced from Uniprot.org

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