ELP1/IKBKAP Antibody

• 产品编号：CST-5071S      品牌：CST       原厂货号：5071S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

ELP1/IKBKAP Antibody能够检测内源性ELP1/IKBKAP总蛋白水平。通过合成的人源ELP1/IKBKAP蛋白羧基端相应的多肽片段去免疫动物从而制备出此多克隆抗体。通过蛋白A和多肽亲和层析纯化获得。

Elongator是一个高度保守的转录延伸因子复合物，它首先在酵母中被鉴定，并且作为高度磷酸化的RNA polymerase II (RNAPII)全酶的部分(1)。Elongator复合物是由6个亚单位ELP1-6组成，并且研究显示有乙酰化转移酶活性 (2)。Elongator乙酰化靶点包含 histone H3和α-tubulin，而histone H3是联系着该复合物的转录延伸功能，以及α-tubulin是与迁移的调节和投射神经元的成熟有关(3-6)。

Elongator的ELP1/IKBKAP亚单位起初被认为作为在NFκB信号通路中的支架蛋白(7)。它包含数个WD40结构域，并且对于Elongator复合物的形成起到关键作用(8)。研究者已经确定ELP1蛋白突变是Familial Dysautonomia (FD)的病因，而该病是一种常染色体隐性退行性神经紊乱(9)。研究认为基于ELP1突变之上Elongator功能缺陷影响数个基因的转录延伸，这些基因涉及细胞运动和神经发育，这可能是FD病人的神经病理学潜在病因(10,11)。

1. Otero, G. et al. (1999) Mol Cell 3, 109-18.
2. Creppe, C. and Buschbeck, M. (2011) J Biomed Biotechnol 2011, 924898.
3. Wittschieben, B.O. et al. (1999) Mol Cell 4, 123-8.
4. Hawkes, N.A. et al. (2002) J Biol Chem 277, 3047-52.
5. Kim, J.H. et al. (2002) Proc Natl Acad Sci USA 99, 1241-6.
6. Creppe, C. et al. (2009) Cell 136, 551-64.
7. Cohen, L. et al. (1998) Nature 395, 292-6.
8. Frohloff, F. et al. (2003) J Biol Chem 278, 956-61.
9. Anderson, S.L. et al. (2001) Am J Hum Genet 68, 753-8.
10. Close, P. et al. (2006) Mol Cell 22, 521-31.
11. Cohen-Kupiec, R. et al. (2011) PLoS One 6, e19147.

原厂资料：

Specificity / Sensitivity

ELP1/IKBKAP Antibody recognizes endogenous levels of total ELP1/IKBKAP protein.

Source / Purification

Polyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to residues near the carboxy terminus of human ELP1/IKBKAP protein. Antibodies are purified by protein A and peptide affinity chromatography.

Background

Elongator is a highly conserved transcription elongation factor complex that was first identified in yeast as part of the hyperphosphorylated RNA polymerase II (RNAPII) holoenzyme (1). The Elongator complex consists of 6 subunits, ELP1-6, and has been shown to have acetyltransferase activity (2). The acetylation targets of Elongator include histone H3, which is linked to the transcription elongation function of the complex, and α-tubulin, which is associated with regulation of migration and maturation of projection neurons (3-6).

The ELP1/IKBKAP subunit of Elongator was initially thought to function as a scaffolding protein within the NFκB signaling pathway (7). It contains several WD40 domains and is critical for the formation of the Elongator complex (8). Investigators have determined that mutations in ELP1 are the cause of Familial Dysautonomia (FD), an autosomal recessive neurodegenerative disorder (9). Research studies have demonstrated that defects in Elongator function upon ELP1 mutation affect transcription elongation of several genes involved in cell motility and neuronal development that may be the underlying cause of the neuropathology of FD patients (10,11).

1. Otero, G. et al. (1999) Mol Cell 3, 109-18.
2. Creppe, C. and Buschbeck, M. (2011) J Biomed Biotechnol 2011, 924898.
3. Wittschieben, B.O. et al. (1999) Mol Cell 4, 123-8.
4. Hawkes, N.A. et al. (2002) J Biol Chem 277, 3047-52.
5. Kim, J.H. et al. (2002) Proc Natl Acad Sci USA 99, 1241-6.
6. Creppe, C. et al. (2009) Cell 136, 551-64.
7. Cohen, L. et al. (1998) Nature 395, 292-6.
8. Frohloff, F. et al. (2003) J Biol Chem 278, 956-61.
9. Anderson, S.L. et al. (2001) Am J Hum Genet 68, 753-8.
10. Close, P. et al. (2006) Mol Cell 22, 521-31.
11. Cohen-Kupiec, R. et al. (2011) PLoS One 6, e19147.

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