DHCR24/Seladin-1 (C59D8) Rabbit mAb

• 产品编号：CST-2033S      品牌：CST       原厂货号：2033S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

DHCR24/Seladin-1 (C59D8)兔单克隆抗体识别内源性的DHCR24/Seladin-1总蛋白水平。该单克隆抗体用与人类DHCR24/Seladin-1蛋白氨基酸序列对应的人工合成肽段免疫动物制成。DHCR24/Seladin-1做为链甾醇症的分子基础被发现（1）。它编码24-脱氢胆固醇还原酶（3β-羟胆固醇Δ-24-还原酶）。这种酶可降低胆固醇的合成中的脱氢胆固醇（1）。链甾醇症中此基因的隐形突变导致酶缺陷和脱氢胆固醇水平升高（1）。DHCR24/Seladin-1被氧化应激诱导，介导活性氧增加导致的Ras诱导的衰老（2）。研究进一步显示DHCR24/Seladin-1水平在氧化应激的急性反应中以胆固醇依赖的方式诱导增加，在氧化反应的慢性反应中以胆固醇依赖的方式减少（3）。另外，过表达携带两个突变而缺乏还原酶活性的DHCR24/Seladin-1会导致细胞在氧化应激中失去保护（3）。这些发现将DHCR24/Seladin-1的还原活性与其在氧化应激中的保护性角色联系起来。此酶还有氢过氧化物清道夫的作用（4）。

1. Waterham, H.R. et al. (2001) Am J Hum Genet 69, 685-94.
2. Wu, C. et al. (2004) Nature 432, 640-5.
3. Kuehnle, K. et al. (2008) Mol Cell Biol 28, 539-50.
4. Lu, X. et al. (2008) Endocrinology 149, 3267-73.

原厂资料：

Specificity / Sensitivity

DHCR24/Seladin-1 (C59D8) Rabbit mAb detects endogenous levels of total DHCR24/Seladin-1 protein.

Source / Purification

Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to the sequence of human DHCR24/Seladin-1.

Background

DHCR24/Seladin-1 was identified as a molecular basis for desmosterolosis (1). It encodes for 24-dehydrocholesterol reductase (3β-hydroxysterol Δ-24-reductase). This enzyme reduces desmosterol in cholesterol biosynthesis (1). Recessive mutations in this gene in desmosterolosis patients lead to a defective enzyme resulting in increased levels of desmosterol (1). DHCR24/Seladin-1 is induced upon oxidative stress and was found to mediate Ras-induced senescence resulting from increased reactive oxygen species (2). Studies further indicate that the level of DHCR24/Seladin-1 is induced in the acute response and reduced in the chronic response to oxidative stress in a cholesterol dependent manner (3). Moreover, overexpression of DHCR24/Seladin-1 bearing two mutations that abolish its reductase acitivity causes the cells to lose protection from oxidative stress (3). These findings thus link the reductase activity of DHCR24/Seladin-1 to its protective role in oxidative stress. This enzyme has also been demonstrated to be a hydrogen peroxide scavenger (4).

1. Waterham, H.R. et al. (2001) Am J Hum Genet 69, 685-94.
2. Wu, C. et al. (2004) Nature 432, 640-5.
3. Kuehnle, K. et al. (2008) Mol Cell Biol 28, 539-50.
4. Lu, X. et al. (2008) Endocrinology 149, 3267-73.

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.