Cell Signaling Technology
XP Monoclonal Antibody

Product Pathways - Translational Control

FXR1 (D10A2) XP® Rabbit mAb #12295

Applications Reactivity Sensitivity MW (kDa) Isotype
W IF-IC H M R Mk Endogenous 78-80, 82-84 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

Specificity / Sensitivity

FXR1 (D10A2) XP® Rabbit mAb recognizes endogenous levels of total FXR1 protein.

Source / Purification

Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Gly574 of human FXR1 protein.

Western Blotting

Western Blotting

Western blot analysis of extracts from various cell lines using FXR1 (D10A2) XP® Rabbit mAb.

Western Blotting

Western Blotting

Western blot analysis of extracts from human and mouse skeletal muscle tissues using FXR1 (D10A2) XP® Rabbit mAb.

IF-IC

IF-IC

Confocal immunofluorescent analysis of C2C12 cells, untreated (left) or MG-132 treated (10 μg/mL, 3 hr; right), using FXR1 (D10A2) XP® Rabbit mAb (green). Actin filaments were labeled with DY-554 phalloidin (red). Blue pseudocolor = DRAQ5® #4084 (fluorescent DNA dye).


Background

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). 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 Argonaute 2 (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. 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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