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Fragile X Mental Retardation Protein 1 (FMR1, FMR-1, FMRP, FRAXA, MGC87458, POF, POF1, Protein FMR-1)

Fragile X Mental Retardation Protein 1 (FMR1, FMR-1, FMRP, FRAXA, MGC87458, POF, POF1, Protein FMR-1)

Cat no: F6072-01

Supplier: United States Biological
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Fragile 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 compo-nent of RNAi effector complexes (RISC), suggesting a role in gene silencing (4). In Drosophila, dFMRP associates with Argonaute2 (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). Applications: Suitable for use in Western Blot. Other applications not tested. Recommended Dilution: Western Blot: 1:1000 Incubate membrane with diluted antibody in 5% w/v BSA, 1X TBS, 0.1% Tween-20 at 4 degrees C with gentle shaking, overnight. Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4 degrees C for short-term only. For long-term storage and to avoid repeated freezing and thawing, add sterile 40-50% glycerol, aliquot and store at -20 degrees C. Aliquots are stable for at least 6 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Catalogue number: F6072-01
Reactivities: Human, Mouse, Rat
Hosts: Rabbit
Applications: Western Blot
Size: 100ul
Form: Supplied as a liquid in 10mM sodium HEPES pH 7.5, 0.1%, 150mM NaCl, 100ug/ml BSA and 50% glycerol.
P type: Pab
Isotype: IgG
Purity: Purified by Protein A affinity chromatography.
References: 1. Verkerk, A.J. et al. (1991) Cell 65, 905-14, 2. Siomi, M.C. et al. (1995) EMBO J 14, 2401-8, 3. Zhang, Y. et al. (1995) EMBO J 14, 5358-66. 4. Caudy, A.A. et al. (2002) Genes Dev 16, 2491-6, 5. Siomi, H. et al. (2004) Ment Retard Dev Disabil Res Rev 10, 68-74.
Additional info: Recongnizes endogenous levels of human total FMRP protein. Species crossreactivity: mouse, rat.

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