{"product_id":"cst-4524s","title":"CST,  4524S, FMRP (G468) Antibody","description":"Polyclonal Antibody for studying FMRP. Validated for Western Blotting. Highly specific and rigorously validated in-house, FMRP (G468) Antibody (CST #4524) is ready to ship.\n\n\u003cb\u003eProduct Usage Information\u003c\/b\u003e\nWestern Blotting: 1:1000\n\u003cb\u003eStorage\u003c\/b\u003e\nSupplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg\/ml BSA and 50% glycerol. Store at -20°C. Do not aliquot the antibody.\n\u003cb\u003eProtocol\u003c\/b\u003e\nAvailable protocols: Western Blotting\n\u003cb\u003eSpecificity \/ Sensitivity\u003c\/b\u003e\nFMRP (G468) Antibody detects endogenous levels of total FMRP protein.\nSpecies Reactivity: Human, Mouse, Rat, Monkey\n\u003cb\u003eSource \/ Purification\u003c\/b\u003e\nPolyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to the sequence around Gly468 of human FMRP protein. Antibodies are purified by protein A and peptide affinity chromatography.\n\u003cb\u003eBackground\u003c\/b\u003e\nFragile X syndrome, a frequent cause of inherited mental retardation, often results from expansion of the CGG trinucleotide repeat in the gene that encodes the fragile X mental retardation protein (FMRP) (1). FMRP (also known as FMR1) and its two autosomal homologs (FXR1 and FXR2) all bind RNA and play a role in the pathogenesis of fragile X syndrome (1-3). Each of these related proteins can associate with one another as well as form homodimers (3). FMRP can act as a translation regulator and is a component of RNAi effector complexes (RISC), suggesting a role in gene silencing (4). In , dFMRP associates with Argonaute 2 (Ago2) and Dicer and coimmunoprecipitates with miRNA and siRNA. These results suggest that fragile X syndrome is related to abnormal translation caused by a defect in RNAi-related pathways (5). In addition, FMRP, FXR1, and FXR2 are components of stress granules (SG) and have been implicated in the translational regulation of mRNAs (6).\n\u003cb\u003eAlternate Names\u003c\/b\u003e\nFMR1; FMRP; FMRP translational regulator 1; fragile X mental retardation 1; Fragile X mental retardation 1 protein; Fragile X mental retardation protein 1; Fragile X messenger ribonucleoprotein 1; FRAXA; MGC87458; POF; POF1; premature ovarian failure 1; Protein FMR-1; Synaptic functional regulator FMR1; truncated FMRP\n\n\u003cb\u003eSpecification\u003c\/b\u003e\n\nREACTIVITY: H M R Mk\nSENSITIVITY: Endogenous\nMW (kDa): 80\nSOURCE: Rabbit","brand":"CST","offers":[{"title":"Default Title","offer_id":46799518630057,"sku":"4524S","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/cst-4524s","provider":"Iright","version":"1.0","type":"link"}