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Fragile X Mental Retardation, Autosomal Homolog 1, L662 (FXR1, Fragile X Mental Retardation Syndrome-related Protein 1, Fragile X Mental Retardation-related Protein 1, hFXR1p)

Fragile X Mental Retardation, Autosomal Homolog 1, L662 (FXR1, Fragile X Mental Retardation Syndrome-related Protein 1, Fragile X Mental Retardation-related Protein 1, hFXR1p)

Cat no: F6072-20C

Supplier: United States Biological
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Fragile X syndrome is a genetic disorder characterized by a spectrum of physical and behavioral features and is a frequent form of inherited mental retardation (1). The X-linked FMRP (FMR-1) and its two autosomal homologs, FXR1 and FXR2, are polyribosome-associated RNA-binding proteins that are involved in the pathogenesis of fragile X syndrome (1-3). Each of the fragile X proteins can self-associate as well as form heteromers with the other two related proteins (3). FMRP can act as a translation regulator and is a component of RNAi effector complexes (RISC), suggesting a role in gene silencing (4). The Drosophila homolog of FMRP (dFMRP) associates with Argonaute2 (Ago2) and Dicer and can coimmunoprecipitate with miRNA and siRNA (5). These results suggest that fragile X syndrome is related to abnormal translation caused by defects in RNAi-related pathways. Applications: Suitable for use in Western Blot. Other applications not tested. Recommended Dilution: Western Blot: 1:1000; Incubate membrane with diluted antibody in 5% 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. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Catalogue number: F6072-20C
Reactivities: Mouse
Hosts: Rabbit
Applications: Western Blot
Size: 100ul
Form: Supplied as a liquid in 10mM sodium HEPES pH 7.5, 150mM sodium chloride,0.1mg/ml BSA and 50% glycerol.
P type: Pab
Isotype: IgG
Purity: Purified by Protein A affinity chromatography.
References: 1. Verkerk, A.J., et al., Cell 65: 905-914 (1991). 2. Siomi, M.C., et al., EMBO J. 14: 2401-2408 (1995). 3. Zhang, Y., et al., EMBO J. 14: 5358-5366 (1995). 4. Caudy, A.A., et al., Genes Dev. 16: 2491-2496 (2002). 5. Siomi, H., et al., Ment. Retard. Dev. Disabil. Res. Rev. 10: 68-74 (2004).
Additional info: Recongnizes endogenous levels of mouse total FXR1 protein.

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