Mitofusin-2 (D1E9) Rabbit mAb

• 产品编号：CST-11925S      品牌：CST       原厂货号：11925S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

Mitofusin-2 (D1E9) Rabbit mAb兔单抗能够检测内源性总线粒体融合蛋白2水平。该单克隆抗体是由合成的人源线粒体融合蛋白2缬氨酸(1573位)肽段免疫动物而制备的。

线粒体融合蛋白是线粒体跨膜GTP酶，在线粒体分裂过程中调控线粒体的融合，对于维护线粒体完整的结构和遗传是必需的（1,2）。在哺乳动物中已经鉴定了两个线粒体融合蛋白，线粒体融合蛋白1和2，它们有60％的氨基酸序列同源性，协同调节线粒体的融合（3）。线粒体融合对于正常细胞生长和发育的重要性已被广泛认（4），可能已经进化为一种机制，以抵消mtDNA突变的有害影响（3）。在小鼠任一线粒体融合蛋白的无效突变都会使胚胎致死，而条件基因敲除的研究表明，在骨骼肌中共同敲除线粒体融合蛋白1和线粒体融合蛋白2，会引进严重的线粒体功能障碍（3）。

研究表明，线粒体融合蛋白2的突变与腓骨肌萎缩症相关，后者是一种逐渐丧失肌肉组织和感官知觉的遗传性神经退行性疾病（5,6）。

1. Zhang, Y. and Chan, D.C. (2007) FEBS Lett 581, 2168-73.
2. Chan, D.C. (2006) Annu Rev Cell Dev Biol 22, 79-99.
3. Chen, H. et al. (2010) Cell 141, 280-9.
4. Bereiter-Hahn, J. and Vöth, M. (1994) Microsc Res Tech 27, 198-219.
5. Kijima, K. et al. (2005) Hum Genet 116, 23-7.
6. Züchner, S. et al. (2004) Nat Genet 36, 449-51.

原厂资料：

Specificity / Sensitivity

Mitofusin-2 (D1E9) Rabbit mAb recognizes endogenous levels of total mitofusin-2 protein.

Source / Purification

Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Val573 of human mitofusin-2 protein.

Background

Mitofusins are mitochondrial transmembrane GTPases that function to regulate mitochondrial fusion, a process that occurs in concert with mitochondrial division and is necessary for the maintenance of structural and genetic mitochondrial integrity (1,2). Two mitofusins have been described in mammals, mitofusin-1 and -2, which share 60% amino acid identity and appear to function coordinately to regulate mitochondrial fusion (3). Mitochondrial fusion is widely recognized as important for normal cell growth and development (4), and may have evolved as a mechanism to offset the deleterious effects of mtDNA mutations (3). Null mutations in either mitofusin are embryonic lethal in mice, whereas conditional knockout studies have shown that combined deletion of mitofusin-1 and mitofusin-2 in skeletal muscle results in severe mitochondrial dysfunction (3).

1. Zhang, Y. and Chan, D.C. (2007) FEBS Lett 581, 2168-73.
2. Chan, D.C. (2006) Annu Rev Cell Dev Biol 22, 79-99.
3. Chen, H. et al. (2010) Cell 141, 280-9.
4. Bereiter-Hahn, J. and Vöth, M. (1994) Microsc Res Tech 27, 198-219.
5. Kijima, K. et al. (2005) Hum Genet 116, 23-7.
6. Züchner, S. et al. (2004) Nat Genet 36, 449-51.

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.
*Species cross-reactivity is determined by western blot.
** Anti-rabbit secondary antibodies must be used to detect this antibody.
Recommended Antibody Dilutions:
Western blotting 1:1000
Immunoprecipitation 1:200
Immunofluorescence (IF-IC) 1:100