Cell Signaling Technology

Product Pathways - Translational Control

FMRP (G468) Antibody #4524

Applications Reactivity Sensitivity MW (kDa) Source
W H M R Endogenous 80 Rabbit

Applications Key:  W=Western Blotting
Reactivity Key:  H=Human  M=Mouse  R=Rat
Species cross-reactivity is determined by western blot. Species enclosed in parentheses are predicted to react based on 100% sequence homology.

Protocols

Specificity / Sensitivity

FMRP (G468) Antibody detects endogenous levels of total FMRP protein.

Source / Purification

Polyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to the sequence around Gly468 of human FMRP protein. Antibodies are purified by protein A and peptide affinity chromatography.

Western Blotting

Western Blotting

Western blot analysis of extracts from various cell lines using FMRP (G468) Antibody.

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

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Companion Products

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

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