验证数据展示
产品信息
86293-1-PBS targets NPC2 in WB, IF/ICC, Indirect ELISA applications and shows reactivity with human, mouse samples.
| 经测试应用 | WB, IF/ICC, Indirect ELISA Application Description |
| 经测试反应性 | human, mouse |
| 免疫原 |
CatNo: Ag13719 Product name: Recombinant human NPC2 protein Source: e coli.-derived, PGEX-4T Tag: GST Domain: 1-151 aa of BC002532 Sequence: MRFLAATFLLLALSTAAQAEPVQFKDCGSVDGVIKEVNVSPCPTQPCQLSKGQSYSVNVTFTSNIQSKSSKAVVHGILMGVPVPFPIPEPDGCKSGINCPIQKDKTYSYLNKLPVKSEYPSIKLVVEWQLQDDKNQSLFCWEIPVQIVSHL 种属同源性预测 |
| 宿主/亚型 | Rabbit / IgG |
| 抗体类别 | Recombinant |
| 产品类型 | Antibody |
| 全称 | Niemann-Pick disease, type C2 |
| 别名 | He1, Human epididymis-specific protein 1, Niemann-Pick disease type C2 protein |
| 计算分子量 | 151 aa, 17 kDa |
| 观测分子量 | 17-21 kDa |
| GenBank蛋白编号 | BC002532 |
| 基因名称 | NPC2 |
| Gene ID (NCBI) | 10577 |
| 偶联类型 | Unconjugated |
| 形式 | Liquid |
| 纯化方式 | Protein A purification |
| UNIPROT ID | P61916 |
| 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
Niemann-Pick Type C (NPC) disease is a lysosomal storage disorder characterized by accumulation of unesterified cholesterol and other lipids in the endolysosomal system. NPC disease results from a defect in either of two distinct cholesterol-binding proteins: a transmembrane protein, NPC1, and a small soluble protein, NPC2. NPC1 or NPC2 deficiency models showed that the function of these two proteins within lysosomes are linked closely. NPC2 is also named human epididymis-specific protein 1 (HE1), defects of which are the cause of Niemann-Pick disease type C2, characterized as a lysosomal storage disorder that affects the viscera and the central nervous system. Recent finding suggests that NPC2 may serve as a novel intracrine/autocrine factor that controls adipocyte differentiation and function as well as potential therapeutic target for the treatment of type 2 diabetes and related metabolic disorders.
