Cell Signaling Technology

Product Pathways - Translational Control

FMRP (D14F4) Rabbit mAb #7104

Applications Reactivity Sensitivity MW (kDa) Isotype
W IF-IC H M R Mk Endogenous 80 Rabbit IgG

Applications Key:  W=Western Blotting  IF-IC=Immunofluorescence (Immunocytochemistry)
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.

Protocols

* 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 Blotting

Western Blotting

Western blot analysis of extracts from various cell lines using FMRP (D14F4) Rabbit mAb.

IF-IC

IF-IC

Confocal immunofluorescent analysis of C2C12 cells, untreated (left) or treated with MG132 (10 μg/mL, 3 hrs; right), using FMRP (D14F4) Rabbit mAb (green). Actin filaments were labeled with DY-554 phalloidin (red). Blue pseudocolor = DRAQ5® #4084 (fluorescent DNA dye).

Background

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.

Application References

Have you published research involving the use of our products? If so we'd love to hear about it. Please let us know!

Companion Products

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

Products