验证数据展示
产品信息
67957-1-PBS targets CLN3 in WB, Indirect ELISA applications and shows reactivity with Human samples.
| 经测试应用 | WB, Indirect ELISA Application Description |
| 经测试反应性 | Human |
| 免疫原 | CLN3 fusion protein Ag31402 种属同源性预测 |
| 宿主/亚型 | Mouse / IgG1 |
| 抗体类别 | Monoclonal |
| 产品类型 | Antibody |
| 全称 | ceroid-lipofuscinosis, neuronal 3 |
| 别名 | Batten disease protein, BATTENIN, BTS, CLN3, JNCL, Protein CLN3 |
| 计算分子量 | 438 aa, 48 kDa |
| 观测分子量 | 50 kDa |
| GenBank蛋白编号 | BC002394 |
| 基因名称 | CLN3 |
| Gene ID (NCBI) | 1201 |
| RRID | AB_2918708 |
| 偶联类型 | Unconjugated |
| 形式 | Liquid |
| 纯化方式 | Protein G purification |
| UNIPROT ID | Q13286 |
| 储存缓冲液 | PBS only , pH 7.3 |
| 储存条件 | Store at -80°C. The product is shipped with ice packs. Upon receipt, store it immediately at -80°C |
背景介绍
Neuronal ceroid lipofuscinosis (NCL, or Batten disease) refers to a group of lethal pediatric neurodegenerative diseases originating from mutations in one of the thus far identified 13 CLN genes (Ceroid Lipofuscinosis, Neuronal type; CLN1 to CLN14) (PMID: 25051496). CLN3 is a multi-membrane spanning protein that is involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes. The CLN3 gene is located on chromosome 16p12.1and produces three mRNA splicing variants. The 438-amino-acid CLN3 protein has a calculated molecular weight of 48 kDa. It has been reported that CLN3 can be glycosylated and form homodimeric complex (PMID: 10356317; 17286803).
