SOD1 Antibody

• 产品编号：CST-2770S      品牌：CST       原厂货号：2770S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

SOD1 Antibody 兔多抗识别内源性的SOD1总蛋白。该多克隆抗体由合成的人类SOD1蛋白氨基末端附近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。SOD1，铜/锌超氧化物歧化酶，是一个重要的抗氧化酶，可催化超氧阴离子自由基转换为过氧化氢与氧分子（1）。SOD1广泛表达，定位于细胞质、细胞核和线粒体膜间隙。SOD1基因位点在21号染色体上可产生唐氏综合症的区域（2）。此外，超过100个不同的SOD1遗传突变在家族性肌萎缩性侧索硬化症（ALS）中被发现，这是一个进行性运动神经元退行性疾病（3-5）。尽管SOD1可帮助消除毒性反应物，其在ALS的突变已被描述为是功能增加性的（5）。突变的SOD1在ALS中诱导神经退行性变的机制目前还不清楚。突变SOD1蛋白会发生错误折叠，并进一步寡聚化成高分子量物质，聚合并最终形成蛋白质包涵体（5）。

1. McCord, J.M. and Fridovich, I. (1988) Free Radic Biol Med 5, 363-9.
2. Sherman, L. et al. (1983) Proc Natl Acad Sci USA 80, 5465-9.
3. Rosen, D.R. et al. (1993) Nature 362, 59-62.
4. Deng, H.X. et al. (1993) Science 261, 1047-51.
5. Valentine, J.S. and Hart, P.J. (2003) Proc Natl Acad Sci USA 100, 3617-22.

原厂资料：

Specificity / Sensitivity

SOD1 Antibody detects endogenous levels of total SOD1 protein.

Source / Purification

Polyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to residues near the amino- terminus of human SOD1. Antibodies were purified by protein A and peptide affinity chromatography.

Background

SOD1, Cu/Zn superoxide dismutase, is a major antioxidant enzyme that catalyzes the conversion of superoxide anion to hydrogen peroxide and molecular oxygen (1). SOD1 is ubiquitously expressed and is localized in the cytosol, nucleus and mitochondrial intermembrane space. The SOD1 gene locus is on chromosome 21 in a region affected in Down Syndrome (2). In addition, over 100 distinct SOD1 inherited mutations have been identified in the familial form of amyotrophic lateral sclerosis (ALS), a progressive degenerative disease of motor neurons (3-5). Despite the fact that SOD1 helps to eliminate toxic reactive species, its mutations in ALS have been described as gain-of-function (5). The mechanism by which mutant SOD1 induces the neurodegeneration observed in ALS is still unclear. Mutant SOD1 proteins become misfolded and consequently oligomerize into high molecular weight species that aggregate and end up in proteinaceous inclusions (5).

1. McCord, J.M. and Fridovich, I. (1988) Free Radic Biol Med 5, 363-9.
2. Sherman, L. et al. (1983) Proc Natl Acad Sci USA 80, 5465-9.
3. Rosen, D.R. et al. (1993) Nature 362, 59-62.
4. Deng, H.X. et al. (1993) Science 261, 1047-51.
5. Valentine, J.S. and Hart, P.J. (2003) Proc Natl Acad Sci USA 100, 3617-22.

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.