Ataxin-1 Antibody

• 产品编号：CST-2177S      品牌：CST       原厂货号：2177S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

Ataxin-1 Antibody 兔多抗识别内源性的总Ataxin-1蛋白。该多克隆抗体由合成的人类Ataxin-1蛋白氨基酸序列对应肽段免疫动物而制成。该抗体使用蛋白A和多肽亲和层析纯化而得。

脊髓小脑性共济失调（SCA1），一种常染色体显性遗传的神经退行性疾病，以口齿不清，肢体协调障碍，步态异常为特征，由于小脑Purkinje细胞和脑干中部分神经元变性导致。（1）SCA1的患者有一个高度多态的CAG重复，其在ataxin-1蛋白中编码一个多聚氨基酸片段（2）。Akt能磷酸化ataxin-1的Ser776位点，后者能够影响其与14-3-3的结合。这种相互作用增加了ataxin-1的稳定性，导致其不断积累，从而诱发神经退行性变化（3）。此外，HSP70可以影响和控制磷酸化对ataxin-1稳定性的作用（4）。

1. Zoghbi, H.Y. and Orr, H.T. (2000) Annu Rev Neurosci 23, 217-47.
2. Orr, H.T. et al. (1993) Nat Genet 4, 221-6.
3. Chen, H.K. et al. (2003) Cell 113, 457-68.
4. Jorgensen, N.D. et al. (2007) J Neurochem 102, 2040-8.

原厂资料：

Specificity / Sensitivity

Ataxin-1 Antibody detects endogenous levels of total ataxin-1 protein.

Source / Purification

Polyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to human ataxin-1. Antibodies are purified by protein A and peptide affinity chromatography.

Background

Spinocerebellar ataxia 1 (SCA1), an autosomal dominant neurodegenerative disorder, is characterized by slurred speech, loss of limb coordination, and gait abnormalities resulting from the degeneration of cerebellar Purkinje cells and of a subset of brainstem neurons (1). Individuals with SCA1 have a highly polymorphic CAG repeat expansion encoding a polyglutamine tract in ataxin-1 (2). Akt phosphorylates ataxin-1 at Ser776, which regulates an association with 14-3-3. This interaction increases ataxin-1 stabilization and accumulation resulting in enhanced neurodegeneration (3). In addition, HSP70 controls the effect that phosphorylation has on ataxin-1 stability (4).

1. Zoghbi, H.Y. and Orr, H.T. (2000) Annu Rev Neurosci 23, 217-47.
2. Orr, H.T. et al. (1993) Nat Genet 4, 221-6.
3. Chen, H.K. et al. (2003) Cell 113, 457-68.
4. Jorgensen, N.D. et al. (2007) J Neurochem 102, 2040-8.

注意事项：

For Research Use Only. Not For Use In Diagnostic Procedures.

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.