ATP2A1/SERCA1 (L24) Antibody

• 产品编号：CST-4219S      品牌：CST       原厂货号：4219S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

ATP2A1/SERCA1(L24)抗体识别内源性的全部ATP2A1/SERCA1蛋白。该多克隆抗体用与人类ATP2A1/SERCA1中Leu24位点附近的氨基酸序列对应的人工合成肽段免疫动物而制成。该抗体使用蛋白A和蛋白亲和层析纯化而得。肌质和内质网Ca2+ ATPases（SERCA）是一个高度保守的Ca2+泵家族成员（1）。SERCA泵将细胞质内Ca2 +沿逆向浓度梯度运输到肌质和内质网（1）。ATP2A1 (SERCA1)是一个快速转换的骨骼肌肌质网Ca2+ATPase（2）。研究表明，ATP2A1基因突变会导致常染色体隐性遗传肌紊乱，就是已知的布罗迪肌病，其特点是运动时伴有肌肉痉挛和肌肉松弛受损（1-3）。

1. Hovnanian, A. (2007) Subcell Biochem 45, 337-63.
2. Odermatt, A. et al. (1996) Nat Genet 14, 191-4.
3. Odermatt, A. et al. (2000) Hum Genet 106, 482-91.

原厂资料：

Specificity / Sensitivity

ATP2A1/SERCA1 (L24) Antibody detects endogenous levels of total ATP2A1/SERCA1 protein.

Source / Purification

Polyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Leu24 of human ATP2A1/SERCA1 protein. Antibodies are purified by protein A and peptide affinity chromatography.

Background

Sarcoplasmic and endoplasmic reticulum Ca2+ ATPases (SERCA) are members of a highly conserved family of Ca2+ pumps (1). SERCA pumps transport Ca2+ from the cytosol to the sarcoplasmic and endoplasmic reticulum lumen against a large concentration gradient (1). ATP2A1 (SERCA1) is a fast-twitch, skeletal muscle sarcoplasmic reticulum Ca2+ ATPase (2). Research studies have shown that mutations in the ATP2A1 gene cause an autosomal recessive muscle disorder known as Brody myopathy, which is characterized by muscle cramping and impaired muscle relaxation associated with exercise (1-3).

1. Hovnanian, A. (2007) Subcell Biochem 45, 337-63.
2. Odermatt, A. et al. (1996) Nat Genet 14, 191-4.
3. Odermatt, A. et al. (2000) Hum Genet 106, 482-91.

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.