| 形态 | Liquid |
|---|---|
| 宿主种属 | Rabbit |
| 克隆性 | Polyclonal |
| 抗体亚型 | IgG |
| 纯化方式 | Purified by antigen affinity column. |
| 内毒素水平 | Please contact with the lab for this information. |
| 应用 | ELISA, IHC, WB |
| 种属反应性 | Human |
| 靶标 | BTS, Batten disease protein, Battenin, CLN3, Protein CLN3 |
| 免疫原 | E. coli - derived recombinant Human CLN3 (Gln204-Val277). |
| 储存缓冲液 | 0.01M PBS, pH 7.4, 50% Glycerol, 0.05% Proclin 300. |
| 稳定性和储存 | Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt. |
| 产品使用说明 | ELISA:1:5000-1:20000;IHC:1:50-1:500;WB:1:500-1:2000 |
| 登录号 | Q13286 |
| 背景信息 | Battenin (CLN3) is a ~47 kDa protein. Mediates microtubule-dependent, anterograde transport connecting the Golgi network, endosomes, autophagosomes, lysosomes and plasma membrane, and participates in several cellular processes such as regulation of lysosomal pH, lysosome protein degradation, receptor-mediated endocytosis, autophagy, transport of proteins and lipids from the TGN, apoptosis and synaptic transmission. Facilitates the proteins transport from trans-Golgi network (TGN)-to other membrane compartments such as transport of microdomain-associated proteins to the plasma membrane, IGF2R transport to the lysosome where it regulates the CTSD release leading to regulation of CTSD maturation and thereby APP intracellular processing. Moreover regulates CTSD activity in response to osmotic stress. Also binds galactosylceramide and transports it from the trans Golgi to the rafts, which may have immediate and downstream effects on cell survival by modulating ceramide synthesis. At the plasma membrane, regulates actin-dependent events including filopodia formation, cell migration, and pinocytosis through ARF1-CDC42 pathway and also the cytoskeleton organization through interaction with MYH10 and fodrin leading to the regulation of the plasma membrane association of Na+, K+ ATPase complex. 1. Golabek, AA. et al. (2000) Molecular genetics and metabolism 70, 203-13. PMID: 10924275 2. Luiro, K. et al. (2004) Human molecular genetics 13, 3017-27. PMID: 15471887 3. Rusyn, E. et al. (2008) Pediatric research 63, 625-31. PMID: 18317235 4. Metcalf, DJ. et al. (2008) Traffic (Copenhagen, Denmark) 9, 1905-14. PMID: 18817525 5. Getty, AL. et al. (2011) Experimental cell research 317, 51-69. PMID: 20850431 6. Uusi-Rauva, K. et al. (2012) Cellular and molecular life sciences : CMLS 69, 2075-89. PMID: 22261744 7. Getty, A. et al. (2013) PloS one 8, e66203. PMID: 23840424 8. Cárcel-Trullols, J. et al. (2017) Journal of cellular biochemistry 118, 3883-3890. PMID: 28390177 |
| 备注 | For research use only |