MTMR14 Antibody

• 产品编号：CST-9057S      品牌：CST       原厂货号：9057S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

MTMR14 Antibody能识别内源性的MTMR14总蛋白。该多克隆抗体通过用合成肽免疫动物制备，该合成肽是人 MTMR14蛋白的Arg500周围的残基相一致。抗体通过蛋白A以及多肽亲和色谱法纯化。肌管素相关蛋白14(MTMR14)，也被称为 Jumpy，是一种肌管素相关的磷酸肌醇-3 - 磷酸(PI3P) 磷酸酶（1）。MTMR14基因的突变与中央核肌病有关（1）。小鼠中MTMR14的缺乏，导致钙稳态改变和肌肉障碍（2）。MTMR14在自噬中也有重要作用，它是通过III型PI3K（Vps34）由磷脂酰肌醇酯高度调控的一个过程（3）。MTMR14主要集中于自噬的隔离膜和早期的自噬体（3）。在这些研究中，MTMR14抑制的自噬和与中央核肌病有关的MTMR14突变在自噬抑制中都有缺陷。在斑马鱼中，MTMR14的下调引起了自噬体数量的增加，说明它的活性与自噬的抑制有关（4）。

1. Tosch, V. et al. (2006) Hum Mol Genet 15, 3098-106.
2. Shen, J. et al. (2009) Nat Cell Biol 11, 769-76.
3. Vergne, I. et al. (2009) EMBO J 28, 2244-58.
4. Gibbs, E.M. et al. (2010) Autophagy 6, 819-20.

原厂资料：

Specificity / Sensitivity

MTMR14 Antibody recognizes endogenous levels of total MTMR14 protein.

Source / Purification

Polyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Arg500 of human MTMR14 protein. Antibodies are purified by protein A and peptide affinity chromatography.

Background

Myotubularin-related protein 14 (MTMR14), also known as Jumpy, is a myotubularin-related phosphoinositol-3-phosphate (PI3P) phosphatase (1). Mutations in the MTMR14 gene have been associated with centronuclear myopathy (1). MTMR14 deficiency in mice leads to altered calcium homeostasis and muscle disorders (2). MTMR14 has also been shown to play a role in autophagy, a process that is highly regulated by phosphatidylinositides through the type III PI3K, Vps34 (3). MTMR14 was localized to autophagic isolation membranes and early autophagosomes (3). In these studies, MTMR14 inhibited autophagy and mutations of MTMR14 associated with centronuclear myopathy were also defective in autophagy inhibition. In zebrafish, MTMR14 knockdown was shown to increase the number of autophagosomes, suggesting that its activity is associated with an inhibition of autophagy (4).

1. Tosch, V. et al. (2006) Hum Mol Genet 15, 3098-106.
2. Shen, J. et al. (2009) Nat Cell Biol 11, 769-76.
3. Vergne, I. et al. (2009) EMBO J 28, 2244-58.
4. Gibbs, E.M. et al. (2010) Autophagy 6, 819-20.

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