Fragile X Mental Retardation, Autosomal Homolog 2 (FXR2, Fragile X Mental Retardation Syndrome Related Protein 2, Fragile X Mental Retardation Gene Autosomal Homolog 2, Fragile X Mental Retardation 1-like 2, FMR1L2)

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.\n\nApplications:\nSuitable for use in Western Blot. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 1:1000. Incubate membrane with diluted antibody in 5% w/v BSA, 1X TBS, 0.1% \n Tween-20 at 4 degrees C with gentle shaking, overnight. \nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. For long-term storage, aliquot and store at -20 degrees C. Aliquots are stable for at least 12 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.

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SPECIFICATIONS

Catalog Number

F6072-30B

Size

100ul

Applications

WB

Hosts

Rabbit

Reactivities

Hum, NHP

Form

Supplied in 10mM sodium HEPES pH 7.5, 150mM NaCl, 100ug/ml BSA and 50% glycerol.

P Type

Pab

Purity

Purified by Protein A affinity chromatography.

Isotype

IgG

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

Recognizes human FXR2. Species crossreactivity: monkey.