TorsinA (D-M2A8) Mouse mAb

• 产品编号：CST-2150S      品牌：CST       原厂货号：2150S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

TorsinA (D-M2A8)Mouse mAb 鼠单抗识别内源性的torsinA 总蛋白。TorsinA (D-M2A8)Mouse mAb 鼠单抗不能识别TorsinB蛋白(Hewett, J. et al., 2004)。该单克隆抗体用重组torsinA (aa 51-324)蛋白免疫动物而制成。

神经系统疾病肌张力障碍是持续的肌肉收缩和姿态障碍（1）。TorsinA，torsinB，torp2A和torp3A属于与细胞活动相关（AAA+）的ATP酶家族，torsinA的突变导致早发性肌张力障碍（2）。TorsinA已被证明能够抑制线虫的细胞内蛋白质聚集，具有分子伴侣活性。有趣的是，torsinA在多巴胺能神经元有高表达，与帕金森病病理特征的Lewy小体中α-突触核蛋白有关（3-5）。

1. Fahn, S. (1988) Adv. Neurol. 50, 1-8.
2. Ozelius, L.J. et al. (1997) Nat. Genet. 17, 40-8.
3. Caldwell, G.A. et al. (2003) Hum. Mol. Genet. 12, 307-19.
4. McLean, P.J. et al. (2002) J. Neurochem. 83, 846-54.
5. O'Farrell, C. et al. (2004) Brain Res. Mol. Brain Res. 127, 1-9.

原厂资料：

Specificity / Sensitivity

TorsinA (D-M2A8) Mouse mAb detects endogenous levels of total torsinA protein. TorsinA (D-M2A8) Mouse mAb does not detect TorsinB (Hewett, J. et al., 2004).

Source / Purification

Monoclonal antibody is produced by immunizing animals with human recombinant torsinA (aa 51-324).

Background

The neurological condition Dystonia is associated with sustained muscle contractions and abnormal posturing (1). TorsinA, torsinB, torp2A and torp3A belong to the family of ATPases associated with cellular activites (AAA+) and mutations in torsinA cause early onset dystonia (2). TorsinA has been shown to suppress intracellular protein aggregation in C. elegans and possesses chaperon activity. Interestingly, torsinA is highly expressed in dopaminergic neurons and associates with alpha-synuclein in Lewy bodies, which pathologically characterize Parkinson's Disease (3-5).

1. Fahn, S. (1988) Adv. Neurol. 50, 1-8.
2. Ozelius, L.J. et al. (1997) Nat. Genet. 17, 40-8.
3. Caldwell, G.A. et al. (2003) Hum. Mol. Genet. 12, 307-19.
4. McLean, P.J. et al. (2002) J. Neurochem. 83, 846-54.
5. O'Farrell, C. et al. (2004) Brain Res. Mol. Brain Res. 127, 1-9.

注意事项：

For Research Use Only. Not For Use In Diagnostic Procedures.

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.