FXR1 (D10A2) XP? Rabbit mAb

• 产品编号：CST-12295P      品牌：Cell Signaling Technology       原厂货号：12295P
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

FXR1 (D10A2) XP® Rabbit mAb能够检测内源性 FXR1总蛋白。该单克隆抗体是经由合成的围绕人 FXR1蛋白 Gly574位点的氨基酸肽段免疫动物而生产的。X染色体易损综合征（Fragile X syndrome）是以一系列生理、行为学特征为特点一种遗传性紊乱，其也是常见的遗传性精神发育迟疾病(1)。X-linked FMRP (FMR-1) 和它的两个常染色体同系物 (FXR1 and FXR2) 都是多核糖体相关的RNA结合蛋白，它们都涉及到fragile X syndrome的发病机制(1-3)。每一个fragile X蛋白都能自我发生联系，也可以与其它两个相关蛋白形成异源二聚体(3)。FMRP能够作为一种翻译调节因子，同时也是RNAi因子复合物(RISC)的组成部分，研究认为该蛋白在基因沉默中发挥作用(4)。在果蝇中，dFMRP与 Argonaute 2 (Ago2)、Dicer和miRNA及siRNA免疫共沉淀物相联系(5)。这些研究结果认为X染色体易损综合征是和由RNAi相关通路的缺陷而引起非正常的蛋白质翻译相关。此外，FMRP、FXR1和FXR2是应激颗粒(SG)组成成员，同时这些蛋白也涉及到mRNAs翻译的调节(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.

原厂资料：

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.

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.

注意事项：

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.