FMRP Antibody

4317:

Immunofluorescence , Immunoprecipitation , Western Blotting

FMRP Antibody detects endogenous levels of total FMRP protein.

Polyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to the sequence of human FMRP. Antibodies are purified by protein A and peptide affinity chromatography.

Western Blotting

Western blot analysis of extracts from various cell lines using FMRP Antibody.

IF-IC

Confocal immunofluorescent analysis of C2C12 cells, untreated or treated with MG-132 (10 μg/mL for 3 hours), using FMRP Antibody (green). Actin filaments were labeled with DY-554 phalloidin (red). Blue pseudocolor = DRAQ5^® #4084 (fluorescent DNA dye).

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 component of RNAi effector complexes (RISC), suggesting a role in gene silencing (4). In Drosophila , 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).

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.
6. Linder, B. et al. (2008) Hum Mol Genet 17, 3236-46.

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For Research Use Only. Not For Use In Diagnostic Procedures.