| 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 | EC:2.7.8.-, FCMD, FKTN, Fukutin, Fukuyama-type congenital muscular dystrophy protein, Ribitol-5-phosphate transferase, Ribitol-5-phosphate transferase FKTN |
| 免疫原 | E. coli - derived recombinant Human FKTN (Cys250-Tyr461). |
| 储存缓冲液 | 0.01M PBS, pH 7.4, 50% Glycerol, 0.05% Proclin 300. |
| 产品使用说明 | ELISA:1:5000-1:20000;IHC:1:50-1:500;WB:1:500-1:2000 |
| 登录号 | O75072 |
| 背景信息 | Ribitol-5-phosphate transferase FKTN is a ~53 kDa protein. Catalyzes the transfer of a ribitol-phosphate from CDP-ribitol to the distal N-acetylgalactosamine of the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1). This constitutes the first step in the formation of the ribitol 5-phosphate tandem repeat which links the phosphorylated O-mannosyl trisaccharide to the ligand binding moiety composed of repeats of 3-xylosyl-alpha-1,3-glucuronic acid-beta-1. Required for normal location of POMGNT1 in Golgi membranes, and for normal POMGNT1 activity. May interact with and reinforce a large complex encompassing the outside and inside of muscle membranes. Could be involved in brain development (Probable). 1. Kanagawa, M. et al. (2016) Cell reports 14, 2209-2223. PMID: 26923585 2. Gerin, I. et al. (2016) Nature communications 7, 11534. PMID: 27194101 3. Nishihara, R. et al. (2018) Biochemical and biophysical research communications 497, 1025-1030. PMID: 29477842 4. Xiong, H. et al. (2006) Biochemical and biophysical research communications 350, 935-41. PMID: 17034757 5. Willer, T. et al. (2014) eLife 3. PMID: 25279699 |
| 备注 | For research use only |