FMRP (G468) Antibody

• 产品编号：CST-4524S      品牌：CST       原厂货号：4524S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

FMRP (G468) Antibody检测内源性FMRP总蛋白水平。通过人工合成人源FMRP蛋白Gly468位点周围序列相应的多肽片段去免疫动物从而制备多克隆抗体。通过蛋白A和多肽亲和层析纯化抗体。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

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.

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.

注意事项：

Storage: Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/ml BSA and 50% glycerol. Store at –20°C. Do not aliquot the antibody.