Fumarase (D9C5) Rabbit mAb

• 产品编号：CST-4567S      品牌：CST       原厂货号：4567S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

Fumarase(D9C5)兔单克隆抗体识别内源性的延胡索酸酶总蛋白水平。该单克隆抗体用与人类延胡索酸酶蛋白中Gly354位点附近的氨基酸序列对应的人工合成肽段免疫动物制成。延胡索酸水合酶（FH）是一个经典的线粒体酶，在三羧酸循环中催化延胡索酸转化为苹果酸（1,2）。研究显示FH缺陷会导致遗传性平滑肌瘤病和肾细胞癌（1,3）或者合子生殖突变及严重神经和肌肉疾病（1,2）。存在细胞质延胡索酸水合酶的同工酶，参与DNA损伤的修复（3）。

1. Isaacs, J.S. et al. (2005) Cancer Cell 8, 143-53.
2. Bourgeron, T. et al. (1994) J Clin Invest 93, 2514-8.
3. Yogev, O. et al. (2010) PLoS Biol 8, e1000328.

原厂资料：

Specificity / Sensitivity

Fumarase (D9C5) Rabbit mAb recognizes endogenous levels of total fumarase protein.

Source / Purification

Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Gly354 of human fumarase protein.

Background

Fumarate hydratase (Fumarase, FH) is canonically regarded as a mitochondrial enzyme of the tricarboxylic acid cycle that catalyzes fumarate to malate (1,2). Studies have demonstrated that deficiencies in FH can lead to hereditary leiomyomatosis and renal cell cancer (1,3) or, in homozygous germline mutations, severe neurological and muscular disorders (1,2). There also exists a cytosolic fumarase isoenzyme that is involved in DNA damage repair (3).

1. Isaacs, J.S. et al. (2005) Cancer Cell 8, 143-53.
2. Bourgeron, T. et al. (1994) J Clin Invest 93, 2514-8.
3. Yogev, O. et al. (2010) PLoS Biol 8, e1000328.

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