验证数据展示
产品信息
26251-1-PBS targets CHM in WB, IHC, IF/ICC, Indirect ELISA applications and shows reactivity with human samples.
| 经测试应用 | WB, IHC, IF/ICC, Indirect ELISA Application Description |
| 经测试反应性 | human |
| 免疫原 |
CatNo: Ag23166 Product name: Recombinant human CHM protein Source: e coli.-derived, PGEX-4T Tag: GST Domain: 69-110 aa of BC063522 Sequence: VSDSPVWQDQILENEEAIALSRKDKTIQHVEVFCYGRSTLLL 种属同源性预测 |
| 宿主/亚型 | Rabbit / IgG |
| 抗体类别 | Polyclonal |
| 产品类型 | Antibody |
| 全称 | choroideremia (Rab escort protein 1) |
| 别名 | Choroideraemia protein, REP1, REP 1, Rab escort protein 1, GGTA |
| 观测分子量 | 95 kDa |
| GenBank蛋白编号 | BC063522 |
| 基因名称 | CHM |
| Gene ID (NCBI) | 1121 |
| RRID | AB_3669530 |
| 偶联类型 | Unconjugated |
| 形式 | Liquid |
| 纯化方式 | Antigen affinity purification |
| UNIPROT ID | P24386 |
| 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
CHM (Choroideremia protein) is also named as Rab escort protein 1 (REP-1), TCD and Rab proteins geranylgeranyltransferase component A 1. Choroideremia (CHM) is an X-linked recessive eye disease that is caused by null mutations in REP-1, a gene involved in the regulation of Rab GTPases and intracellular vesicular transport (PMID: 9067750, PMID: 16936131). REP-1 is involved in trafficking of Rab proteins in the cell. And REP-1 binds newly synthesized Rab proteins and presents them to the catalytic site of RabGGTase (PMID: 19427510). RAB-27 in several neurons, such as motor neurons at neuromuscular junctions, requires REP-1 for normal synaptic transmission responsible for aldicarb sensitivity (PMID: 19090809). REP-1 acts as a component of the protein complex. There are two similar Rab escort proteins (75% sequence identity): REP-1 and REP-2. A 95-kDa protein (previously designated component A and now called REP-1) is present (PMID: 8294464, PMID: 17140818).


