Product Pathways - Translational Control
FMRP (D14F4) Rabbit mAb #7104
|W IF-IC||H M R Mk||Endogenous||80||Rabbit IgG|
Reactivity Key: H=Human M=Mouse R=Rat Mk=Monkey
Species cross-reactivity is determined by western blot. Species enclosed in parentheses are predicted to react based on 100% sequence homology.
* Product-specific protocol.
Specificity / Sensitivity
FMRP (D14F4) Rabbit mAb recognizes endogenous levels of total FMRP protein.
Source / Purification
Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Gly552 of human FMRP protein.
Western blot analysis of extracts from various cell lines using FMRP (D14F4) Rabbit mAb.
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).
- Verkerk, A.J. et al. (1991) Cell 65, 905-14.
- Siomi, M.C. et al. (1995) EMBO J 14, 2401-8.
- Zhang, Y. et al. (1995) EMBO J 14, 5358-66.
- Caudy, A.A. et al. (2002) Genes Dev 16, 2491-6.
- Siomi, H. et al. (2004) Ment Retard Dev Disabil Res Rev 10, 68-74.
- 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.