Description
Product Usage Information
Western Blotting: 1:1000
Immunofluorescence (Immunocytochemistry): 1:50
Storage
Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/ml BSA, 50% glycerol and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody.
Protocol
Available protocols: Western Blotting, Immunofluorescence (Immunocytochemistry)
Specificity / Sensitivity
FXR1 (D10A2) Rabbit Monoclonal Antibody recognizes endogenous levels of total FXR1 protein.
Species Reactivity: Human, Mouse, Rat, Monkey
Source / Purification
Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Gly574 of human FXR1 protein.
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 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).
Alternate Names
FMR1 autosomal homolog 1; Fragile X mental retardation syndrome-related protein 1; fragile X mental retardation, autosomal homolog 1; FXR1; FXR1P; hFXR1p; MYOPMIL; MYORIBF; RNA-binding protein FXR1
