Succinyl-CoA Synthetase Antibody

• 产品编号：CST-5557S      品牌：CST       原厂货号：5557S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

Succinyl-CoA Synthetase Rabbit mAb用于检测内源性琥珀酰辅酶A合成酶(α亚基)总蛋白水平。该多抗通过用与人源蛋琥珀酰辅酶A合成酶α 亚基N端序列相同的合成肽免疫动物制备而来。琥珀酰辅酶A合成酶α亚基(SUCLG1) 催化将琥珀酸转变成琥珀酰辅酶A的化学反应，此反应在柠檬酸循环中发挥重要作用(1,2)。该酶的缺乏会导致多种疾病，如致死性婴儿乳酸中毒和线粒体脑肌病(4)。

1. Johnson, J.D. et al. (1998) J Biol Chem 273, 27580-6.
2. Phillips, D. et al. (2009) Biochemistry 48, 7140-9.
3. Ostergaard, E. et al. (2007) Am J Hum Genet 81, 383-7.
4. Van Hove, J.L. et al. (2010) Pediatr Res 68, 159-64.

原厂资料：

Specificity / Sensitivity

Succinyl-CoA Synthetase Antibody detects endogenous levels of total succinyl-CoA synthetase (α subunit) protein.

Source / Purification

Polyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to residues near the amino terminus of human succinyl-CoA synthetase (α subunit) protein. Antibodies are purified by protein A and peptide affinity chromatography.

Background

Succinyl-CoA synthetase α subunit (SUCLG1) catalyzes the conversion of succinate to succinyl-CoA and plays a key role in the citric acid cycle (1,2). Deficiency of this enzyme leads to a variety of diseases including fatal infantile lactic acidosis (3) and mitochondrial hepatoencephalomyopathy (4).

1. Johnson, J.D. et al. (1998) J Biol Chem 273, 27580-6.
2. Phillips, D. et al. (2009) Biochemistry 48, 7140-9.
3. Ostergaard, E. et al. (2007) Am J Hum Genet 81, 383-7.
4. Van Hove, J.L. et al. (2010) Pediatr Res 68, 159-64.

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